NEJM â Collection Updates for Neurologic Disease

CLINICAL PRACTICE: Jet Lag

Robert L. Sack, M.D. — Thu, 04Â FebÂ 2010 00:00:00 -05:00

A 55-year-old physician is planning a trip from Los Angeles to London to attend a scientific conference. His previous trip to Europe was complicated by sleepiness during meetings and difficulty...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 3-2010 â A 5-Month-Old Boy with Developmental Delay and Irritability

Thu, 28Â JanÂ 2010 00:00:00 -05:00

A 4.5-month-old boy was seen in the pediatric neurology clinic of this hospital because of developmental delay and an abnormal neurologic examination.

The patient had been born at full-term by normal...

REVIEW ARTICLE: Alzheimer's Disease

Henry W. Querfurth, M.D., Ph.D., and Frank M. LaFerla, Ph.D. — Thu, 28Â JanÂ 2010 00:00:00 -05:00

More than 35 million people worldwide â 5.5 million in the United States â have Alzheimer's disease, a deterioration of memory and other cognitive domains that leads to death within...

CORRESPONDENCE: Lovastatin in X-Linked Adrenoleukodystrophy

Thu, 21Â JanÂ 2010 00:00:00 -05:00

To the Editor: As reported previously in the Journal, lovastatin lowers levels of very-long-chain fatty acids in plasma in patients with X-linked adrenoleukodystrophy (X-ALD).1 Further studies did not reproduce this...

REVIEW ARTICLE: Nosocomial Bacterial Meningitis

Thu, 14Â JanÂ 2010 00:00:00 -05:00

Nosocomial bacterial meningitis may result from invasive procedures (e.g., craniotomy, placement of internal or external ventricular catheters, lumbar puncture, intrathecal infusions of medications, or spinal anesthesia), complicated head trauma, or...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Gene Therapy for Color Blindness

Jean Bennett, M.D., Ph.D. — Thu, 17Â DecÂ 2009 00:00:00 -05:00

The inability to distinguish particular color combinations, or âcolor blindness,â is one of the most common and best understood inherited anomalies, having first been self-diagnosed more than 200 years ago...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 38-2009 â A 16-Year-Old Boy with Paroxysmal Headaches and Visual Changes

Thu, 10Â DecÂ 2009 00:00:00 -05:00

A 16-year-old boy was seen in the pediatric neurology clinic of this hospital because of paroxysmal headaches and visual changes.

The patient had been well until approximately 4 months earlier, when...

IMAGES IN CLINICAL MEDICINE: Blurred Vision after Cardiac Catheterization

Carsten H. Meyer, M.D. and Frank G. Holz, M.D. — Thu, 10Â DecÂ 2009 00:00:00 -05:00

A 56-year-old woman with hypertension presented with a sudden onset of painless impaired vision in the right eye. On the previous day, she had undergone cardiac catheterization for evaluation of...

CLINICAL PROBLEM-SOLVING: A Crisis in Late Pregnancy

Thu, 03Â DecÂ 2009 00:00:00 -05:00

A 31-year-old woman in the 37th week of an uncomplicated pregnancy presented to the emergency department with sudden onset of severe bitemporal headache and shortness of breath. Her medical history...

ORIGINAL ARTICLE: A Novel Protective Prion Protein Variant that Colocalizes with Kuru Exposure

Thu, 19Â NovÂ 2009 00:00:00 -05:00

Background: Kuru is a devastating epidemic prion disease that affected a highly restricted geographic area of the Papua New Guinea highlands; at its peak, it predominantly affected adult women and...

INTERACTIVE MEDICAL CASE: A Crisis in Late Pregnancy

Thu, 12Â NovÂ 2009 00:00:00 -05:00

A 31-year-old woman in the 37th week of pregnancy presented to the emergency room of another hospital with sudden onset of severe bitemporal headache and shortness of breath. She was...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 35-2009 â A 60-Year-Old Male Renal-Transplant Recipient with Renal Insufficiency, Diabetic Ketoacidosis, and Mental-Status Changes

Thu, 12Â NovÂ 2009 00:00:00 -05:00

Dr. Peter P. Moschovis (Medicine and Pediatrics): A 60-year-old man with diabetes mellitus and a history of renal transplantation was admitted to this hospital because of mental-status changes, diarrhea, renal...

SPECIAL ARTICLE: Outcome Reporting in Industry-Sponsored Trials of Gabapentin for Off-Label Use

Thu, 12Â NovÂ 2009 00:00:00 -05:00

Background: There is good evidence of selective outcome reporting in published reports of randomized trials.

Methods: We examined reporting practices for trials of gabapentin funded by Pfizer and Warner-Lambert's subsidiary, Parke-Davis...

ORIGINAL ARTICLE: On-Pump versus Off-Pump Coronary-Artery Bypass Surgery

Thu, 05Â NovÂ 2009 00:00:00 -05:00

Background: Coronary-artery bypass grafting (CABG) has traditionally been performed with the use of cardiopulmonary bypass (on-pump CABG). CABG without cardiopulmonary bypass (off-pump CABG) might reduce the number of complications related...

IMAGES IN CLINICAL MEDICINE: Central Nervous System Hemorrhage

Joshua P. Klein, M.D., Ph.D. and Robin C. Ryther, M.D., Ph.D. — Thu, 29Â OctÂ 2009 00:00:00 -05:00

A 68-year-old man who had hypertension, diabetes, and atrial fibrillation and was being treated with warfarin was discovered snoring and unarousable at home. He had been in his usual state...

PERSPECTIVE: When Diseases Disappear â The Case of Familial Dysautonomia

Thu, 22Â OctÂ 2009 00:00:00 -05:00

The success of genetic-screening programs raises an intriguing possibility: some dread diseases of the 20th century may soon become history. A representative example is familial dysautonomia, a severe neurologic condition,...

IMAGES IN CLINICAL MEDICINE: Keratoconus Complicated by Acute Corneal Hydrops

Hiroko Suzuki, M.D. and John Robert Minarcik, M.D. — Thu, 15Â OctÂ 2009 00:00:00 -05:00

A 21-year-old woman with a history of myopia presented with the acute onset of foggy vision in her right eye and associated photophobia and mild sensation of the presence of...

EDITORIAL: Dying from Dementia

Greg A. Sachs, M.D. — Thu, 15Â OctÂ 2009 00:00:00 -05:00

As a teenager, I had the unfortunate but ultimately career-shaping experience of watching my maternal grandmother decline from Alzheimer's disease. She resided in a nursing home, where her final months...

IMAGES IN CLINICAL MEDICINE: Living with One Hemisphere â A Large Porencephalic Cyst

Antonio Trabacca, M.D. and Franca DiCuonzo, M.D. — Thu, 15Â OctÂ 2009 00:00:00 -05:00

A 2-year-old girl presented with gait disturbance and difficulty using her right hand. She had been born by normal spontaneous delivery at 38 weeks of gestation after an uncomplicated pregnancy....

ORIGINAL ARTICLE: The Clinical Course of Advanced Dementia

Thu, 15Â OctÂ 2009 00:00:00 -05:00

Background: Dementia is a leading cause of death in the United States but is underrecognized as a terminal illness. The clinical course of nursing home residents with advanced dementia has...

IMAGES IN CLINICAL MEDICINE: Cerebrospinal Fluid Leak

Theo Diekman, M.D., Ph.D. and Bram Mees, M.D. — Thu, 01Â OctÂ 2009 00:00:00 -05:00

A 50-year-old postmenopausal woman with asthma and allergic rhinitis presented with a 6-month history of unilateral rhinorrhea. When she woke in the mornings, she noticed a stain (halo) on her...

ORIGINAL ARTICLE: Moderate Hypothermia to Treat Perinatal Asphyxial Encephalopathy

Thu, 01Â OctÂ 2009 00:00:00 -05:00

Background: Whether hypothermic therapy improves neurodevelopmental outcomes in newborn infants with asphyxial encephalopathy is uncertain.

Methods: We performed a randomized trial of infants who were less than 6 hours of age...

ORIGINAL ARTICLE: Treatment of Medulloblastoma with Hedgehog Pathway Inhibitor GDC-0449

Thu, 17Â SepÂ 2009 00:00:00 -05:00

Medulloblastoma is the most common malignant brain tumor in children. Aberrant activation of the hedgehog signaling pathway is strongly implicated in the development of some cases of medulloblastoma. A 26-year-old...

ORIGINAL ARTICLE: Progressive Multifocal Leukoencephalopathy after Natalizumab Monotherapy

Thu, 10Â SepÂ 2009 00:00:00 -05:00

We describe progressive multifocal leukoencephalopathy (PML) caused by infection with human polyomavirus JC virus in a patient with multiple sclerosis who was treated with natalizumab. The first PML symptoms appeared...

ORIGINAL ARTICLE: Treatment of Progressive Multifocal Leukoencephalopathy Associated with Natalizumab

Thu, 10Â SepÂ 2009 00:00:00 -05:00

We describe the clinical and therapeutic course of a 52-year-old patient with multiple sclerosis in whom progressive multifocal leukoencephalopathy (PML) developed after 12 months of therapy with natalizumab. The patient...

ORIGINAL ARTICLE: Asymptomatic Reactivation of JC Virus in Patients Treated with Natalizumab

Thu, 10Â SepÂ 2009 00:00:00 -05:00

Background: Progressive multifocal leukoencephalopathy (PML) occurs in a fraction of patients with multiple sclerosis who were treated with natalizumab. Most adults who are infected with the JC virus, the etiologic...

PERSPECTIVE: Reemergence of PML in Natalizumab-Treated Patients â New Cases, Same Concerns

Thu, 10Â SepÂ 2009 00:00:00 -05:00

When progressive multifocal leukoencephalopathy (PML), a rare demyelinating disease induced by JC virus, appeared in 2004 as a complication of natalizumab treatment for multiple sclerosis, a more common demyelinating disease,...

CORRESPONDENCE: Renal Sympathetic-Nerve Ablation for Uncontrolled Hypertension

Thu, 27Â AugÂ 2009 00:00:00 -05:00

To the Editor: The renal sympathetic nerves have been identified as a major contributor to the complex pathophysiology of hypertension in both experimental models and in humans.1 Patients with essential...

IMAGES IN CLINICAL MEDICINE: Retinal Detachment in Malignant Hypertension

Iqbal Tajunisah, F.R.C.S. and Dinesh Kumar Patel, M.S. (Ophthal.) — Thu, 27Â AugÂ 2009 00:00:00 -05:00

A 37-year-old woman with no previous medical conditions presented with a 3-day history of headache and blurry vision. Her blood pressure was elevated, at 220/150 mm Hg, and visual acuity...

CORRESPONDENCE: Vision 1 Year after Gene Therapy for Leber's Congenital Amaurosis

Thu, 13Â AugÂ 2009 00:00:00 -05:00

To the Editor: Leber's congenital amaurosis, a common cause of blindness in infants and children,1 recently became the first human genetic retinal disease to show improved vision in response to...

CORRESPONDENCE: Spinal Injuries in the Sichuan Earthquake

Thu, 06Â AugÂ 2009 00:00:00 -05:00

To the Editor: A magnitude 8.0 earthquake hit the densely populated region of Sichuan, China, on May 12, 2008, causing an estimated 374,643 injuries. By July 23, 2008, a total...

CORRESPONDENCE: Mirror Therapy for Chronic Complex Regional Pain Syndrome Type 1 and Stroke

Thu, 06Â AugÂ 2009 00:00:00 -05:00

To the Editor: Complex regional pain syndrome type 1 is characterized by pain, sensory disturbances (e.g., pain evoked by light brushing of the skin [allodynia]), motor impairment (e.g., weakness), and...

CLINICAL PRACTICE: Neurologic Prognosis after Cardiac Arrest

G. Bryan Young, M.D. — Thu, 06Â AugÂ 2009 00:00:00 -05:00

A 55-year-old man collapses while jogging through the park. A bystander finds him unconscious and without a pulse and initiates cardiopulmonary resuscitation (CPR) while an ambulance is summoned. On arrival...

CORRESPONDENCE: Whole-Body Magnetic Resonance Neurography

Tomohiro Yamashita, M.D., Thomas C. Kwee, M.D., and Taro Takahara, M.D., Ph.D. — Thu, 30Â JulÂ 2009 00:00:00 -05:00

To the Editor: Noninvasive selective visualization of the whole peripheral nervous system may be useful but has not been possible. Although the brain and spinal cord are well visualized with...

ORIGINAL ARTICLE: AGC1 Deficiency Associated with Global Cerebral Hypomyelination

Thu, 30Â JulÂ 2009 00:00:00 -05:00

The mitochondrial aspartateâglutamate carrier isoform 1 (AGC1), specific to neurons and muscle, supplies aspartate to the cytosol and, as a component of the malateâaspartate shuttle, enables mitochondrial oxidation of cytosolic...

IMAGES IN CLINICAL MEDICINE: Myotonic Dystrophy

Luis M. Franco, M.D. and Josh C. Skorupski, M.D. — Thu, 23Â JulÂ 2009 00:00:00 -05:00

A 36-year-old woman was seen in the clinic after congenital myotonic dystrophy was diagnosed in her newborn son. The neonate had profound hypotonia, areflexia, and bradycardia and required mechanical ventilation....

CORRESPONDENCE: Muscle Glycogenosis Due to Phosphoglucomutase 1 Deficiency

Thu, 23Â JulÂ 2009 00:00:00 -05:00

To the Editor: Muscle glycogen storage diseases are rare inborn diseases caused by errors of metabolism associated with either dynamic, exercise-related symptoms or permanent muscle weakness. The most common glycogen...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 23-2009 â A 13-Year-Old Boy with Headache, Nausea, Seizures, and Hypertension

Thu, 23Â JulÂ 2009 00:00:00 -05:00

A 13-year-old boy was admitted to the hospital because of headache, nausea, seizures, renal failure, and hypertension.

The patient had been well until approximately 3 weeks earlier, when intermittent left-sided headaches...

ORIGINAL ARTICLE: Hearing Improvement after Bevacizumab in Patients with Neurofibromatosis Type 2

Thu, 23Â JulÂ 2009 00:00:00 -05:00

Background: Profound hearing loss is a serious complication of neurofibromatosis type 2, a genetic condition associated with bilateral vestibular schwannomas, benign tumors that arise from the eighth cranial nerve. There...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Memory and the NMDA Receptors

Fei Li, M.D., Ph.D., and Joe Z. Tsien, Ph.D. — Thu, 16Â JulÂ 2009 00:00:00 -05:00

The N-methyl-D-aspartate (NMDA) receptor (NMDAR) is the predominant molecular device for controlling synaptic plasticity and memory function.1 Thus, an understanding of the control and action of the NMDAR at central...

EDITORIAL: Effect of Genetic Testing for Risk of Alzheimer's Disease

Rosalie A. Kane, Ph.D., and Robert L. Kane, M.D. — Thu, 16Â JulÂ 2009 00:00:00 -05:00

Genetic testing can be considered a complex variant of diagnostic testing. If the results are not actionable, the findings may lead to anxiety or even life-disrupting actions with little offsetting...

ORIGINAL ARTICLE: Longitudinal Modeling of Age-Related Memory Decline and the Îµ4 Effect

Thu, 16Â JulÂ 2009 00:00:00 -05:00

Background: The APOE Îµ4 allele is associated with the risk of late-onset Alzheimer's disease. The age at which memory decline diverges among persons who are homozygous for the APOE Îµ4...

ORIGINAL ARTICLE: Disclosure of Genotype for Risk of Alzheimer's Disease

Thu, 16Â JulÂ 2009 00:00:00 -05:00

Background: The apolipoprotein E (APOE) genotype provides information on the risk of Alzheimer's disease, but the genotyping of patients and their family members has been discouraged. We examined the effect...

IMAGES IN CLINICAL MEDICINE: Traumatic Bleb

Demetrios Vavvas, M.D., Ph.D. and Katarzyna Brodowska, M.D. — Thu, 09Â JulÂ 2009 00:00:00 -05:00

A 38-year-old man was referred for evaluation of a large, pigmented lesion in the left eye for possible uveal melanoma. The patient reported that the growth had developed during the...

ORIGINAL ARTICLE: Lack of Association between Folate-Receptor Autoantibodies and Neural-Tube Defects

Thu, 09Â JulÂ 2009 00:00:00 -05:00

Background: A previous report described the presence of autoantibodies against folate receptors in 75% of serum samples from women with a history of pregnancy complicated by a neural-tube defect, as...

EDITORIAL: Diabetes Complications and the ReninâAngiotensin System

Thu, 02Â JulÂ 2009 00:00:00 -05:00

The hypothesis that inhibition of the reninâangiotensin system may be effective in preventing diabetic nephropathy was based on a large body of evidence.1 Positive findings from studies in animal models...

REVIEW ARTICLE: Rhabdomyolysis and Acute Kidney Injury

Thu, 02Â JulÂ 2009 00:00:00 -05:00

Rhabdomyolysis â literally, the dissolution of striped (skeletal) muscle â is characterized by the leakage of muscle-cell contents, including electrolytes, myoglobin, and other sarcoplasmic proteins (e.g., creatine kinase, aldolase, lactate...

ORIGINAL ARTICLE: Renal and Retinal Effects of Enalapril and Losartan in Type 1 Diabetes

Thu, 02Â JulÂ 2009 00:00:00 -05:00

Background: Nephropathy and retinopathy remain important complications of type 1 diabetes. It is unclear whether their progression is slowed by early administration of drugs that block the reninâangiotensin system.

Methods: We...

BOOK REVIEW:

Thu, 02Â JulÂ 2009 00:00:00 -05:00

Seeing the three-dimensional world with two-dimensional retinas presents the brain with two problems. To locate an object in space requires depth perception, which can be acquired from one eye alone...

ORIGINAL ARTICLE: Treating Childhood Acute Lymphoblastic Leukemia without Cranial Irradiation

Thu, 25Â JunÂ 2009 00:00:00 -05:00

Background: Prophylactic cranial irradiation has been a standard treatment in children with acute lymphoblastic leukemia (ALL) who are at high risk for central nervous system (CNS) relapse.

Methods: We conducted a...

IMAGES IN CLINICAL MEDICINE: Combined-System Disease

Carl B. Heilman, M.D. and Vasilios A. Zerris, M.D., M.P.H. — Thu, 18Â JunÂ 2009 00:00:00 -05:00

A 76-year-old woman presented with a 3-month history of bilateral numbness and tingling in her hands and feet. She also noted tingling along her spine on forward flexion of the...

BOOK REVIEW:

Thu, 11Â JunÂ 2009 00:00:00 -05:00

In these exciting times of nanotechnology, the neuromuscular junction is an interesting example of a remarkably robust and efficient small machine. One normal nerve impulse opens about 200 presynaptic vesicles,...

BOOK REVIEW:

Thu, 04Â JunÂ 2009 00:00:00 -05:00

The mystery and complexity of autism lead many to use metaphor in their attempts to describe it. Bear with me as I do the same. These two books lead the...

EDITORIAL: Cool with Plaques and Tangles

Douglas C. Ewbank, Ph.D., and Steven E. Arnold, M.D. — Thu, 28Â MayÂ 2009 00:00:00 -05:00

Survivors to the age of 95 years are a select and hardy few. At current mortality rates, only about 8% of persons will live to the age of 95, and...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 17-2009 â A 30-Year-Old Man with Progressive Neurologic Deficits

Thu, 28Â MayÂ 2009 00:00:00 -05:00

Dr. Katharina Busl (Neurology): A 30-year-old man was admitted to this hospital because of progressive neurologic deficits.

Approximately 1 year before this admission, he began to have intermittent headaches and the...

ORIGINAL ARTICLE: Age, Neuropathology, and Dementia

Thu, 28Â MayÂ 2009 00:00:00 -05:00

Background: Research in Alzheimer's disease is focused mainly on younger old persons, whereas studies involving very old persons report attenuated relationships between the pathological features of Alzheimer's disease and dementia.

Methods:...

IMAGES IN CLINICAL MEDICINE: Patulous Eustachian Tube and Eustachian-Tube Dysfunction

Rodney C. Diaz, M.D. — Thu, 21Â MayÂ 2009 00:00:00 -05:00

A 36-year-old woman presented with a 4-year history of intermittent bilateral aural fullness, subjective hearing loss, and popping and crackling tinnitus with autophony of the left ear. Aural symptoms were...

BOOK REVIEW:

Thu, 21Â MayÂ 2009 00:00:00 -05:00

Disorders of the brain have long inspired the literary imagination. Kirsten Menger-Anderson's novel, Doctor Olaf van Schuler's Brain, is a recent addition to this literature. Menger-Anderson traces more than 300...

IMAGES IN CLINICAL MEDICINE: Bull's-Eye Maculopathy

Hugo Mesquita Nogueira, M.D. and Rita Dinis Gama, M.D. — Thu, 21Â MayÂ 2009 00:00:00 -05:00

A 64-year-old woman with systemic lupus erythematosus and normal renal function had received chloroquine at a dose of 3.24 mg per kilogram of lean body weight per day for 6...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 15-2009 â A 25-Year-Old Man with Coma after Cardiac Arrest

Thu, 14Â MayÂ 2009 00:00:00 -05:00

Dr. Mathias Lichterfeld (Infectious Disease): A 25-year-old man was admitted to this hospital because of coma after cardiac arrest.

He had been well until 4 hours before admission, when he was...

ORIGINAL ARTICLE: Fatal Case of Deer Tick Virus Encephalitis

Thu, 14Â MayÂ 2009 00:00:00 -05:00

Deer tick virus is related to Powassan virus, a tickborne encephalitis virus. A 62-year-old man presented with a meningoencephalitis syndrome and eventually died. Analyses of tissue samples obtained during surgery...

ORIGINAL ARTICLE: Antivenom for Critically Ill Children with Neurotoxicity from Scorpion Stings

Thu, 14Â MayÂ 2009 00:00:00 -05:00

Background: Clinically significant scorpion envenomation by Centruroides sculpturatus produces a dramatic neuromotor syndrome and respiratory insufficiency that often necessitate intensive supportive care. We hypothesized that a scorpion-specific F(abâ²)2 antivenom would...

BOOK REVIEW:

Thu, 07Â MayÂ 2009 00:00:00 -05:00

This is not a straightforward textbook on Huntington's disease, a condition that causes much distress to patients and their relatives by virtue of the combination of cognitive decline and involuntary...

BOOK REVIEW:

Thu, 07Â MayÂ 2009 00:00:00 -05:00

Joseph Babinski (1857â1932) â recognized today primarily for the eponymous Babinski sign of the physical examination â became a pioneer of modern neurology when he broke with the tradition of...

EDITORIAL: Membrane Physiology â Bridging the Gap between Medical Disciplines

Markus Bleich, M.D. — Thu, 07Â MayÂ 2009 00:00:00 -05:00

The general separation between medical disciplines has, for decades, been a strange tradition. For example, neurologists and nephrologists have each had their defined patient groups, and many are barely interested,...

CLINICAL PROBLEM-SOLVING: A Red Flag

Thu, 07Â MayÂ 2009 00:00:00 -05:00

A 64-year-old man was brought to the emergency department because of sudden-onset blurred vision in both eyes and diplopia, as well as a frontal headache that had begun earlier that...

ORIGINAL ARTICLE: Epilepsy, Ataxia, Sensorineural Deafness, Tubulopathy, and Mutations

Thu, 07Â MayÂ 2009 00:00:00 -05:00

Background: Five children from two consanguineous families presented with epilepsy beginning in infancy and severe ataxia, moderate sensorineural deafness, and a renal salt-losing tubulopathy with normotensive hypokalemic metabolic alkalosis. We...

IMAGES IN CLINICAL MEDICINE: Chvostek's Sign and Carpopedal Spasm

Ganesh Athappan, M.D. and Venkatesh Kumar Ariyamuthu, M.D. — Thu, 30Â AprÂ 2009 00:00:00 -05:00

A 35-year-old man presented with a 2-day history of cramps and paresthesias in the arms, predominantly involving the fingers. This presentation was preceded by a bout of viral gastroenteritis 1...

IMAGES IN CLINICAL MEDICINE: Bilateral Subacute Subdural Hematomas

Joshua J. Wind, M.D. and James W. Leiphart, M.D., Ph.D. — Thu, 23Â AprÂ 2009 00:00:00 -05:00

A 43-year-old man presented to the emergency department with a 2-day history of headache; he reported being otherwise healthy. Four weeks earlier, he had been an unrestrained driver involved in...

CORRESPONDENCE: Mutations in a Thiamine-Transporter Gene and Wernicke's-like Encephalopathy

Thu, 23Â AprÂ 2009 00:00:00 -05:00

To the Editor: We report on two previously healthy Japanese brothers with a newly discovered recessively inherited syndrome similar to Wernicke's encephalopathy that developed in the second decade of life;...

ORIGINAL ARTICLE: Association of HTRA1 Mutations and Familial Ischemic Cerebral Small-Vessel Disease

Thu, 23Â AprÂ 2009 00:00:00 -05:00

Background: The genetic cause of cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL), which is characterized by ischemic, nonhypertensive, cerebral small-vessel disease with associated alopecia and spondylosis, is...

EDITORIAL: Which Drug for the Pregnant Woman with Epilepsy?

TorbjÃ¶rn Tomson, M.D. — Thu, 16Â AprÂ 2009 00:00:00 -05:00

Approximately 25,000 children are born in the United States each year to mothers with epilepsy.1 Most of these women need to continue taking medication during pregnancy, since uncontrolled seizures may...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 12-2009 â A 46-Year-Old Man with Migraine, Aphasia, and Hemiparesis and Similarly Affected Family Members

Thu, 16Â AprÂ 2009 00:00:00 -05:00

A 46-year-old man was seen in the neurology clinic because of hemiparesis, aphasia, and abnormalities on neuroimaging studies.

The patient had been well, except for migraine headaches, until 4 years earlier,...

IMAGES IN CLINICAL MEDICINE: Keyhole Pupils

Smita I. Negi, M.D. and Guha Krishnaswamy, M.D. — Thu, 16Â AprÂ 2009 00:00:00 -05:00

A 60-year-old man was evaluated for nasal congestion and ocular itchiness that had been ongoing for several years. Examination was unremarkable, showing normal visual acuity, except for symmetric defects in...

ORIGINAL ARTICLE: Cognitive Function at 3 Years of Age after Fetal Exposure to Antiepileptic Drugs

Thu, 16Â AprÂ 2009 00:00:00 -05:00

Background: Fetal exposure of animals to antiepileptic drugs at doses lower than those required to produce congenital malformations can produce cognitive and behavioral abnormalities, but cognitive effects of fetal exposure...

PERSPECTIVE: Care of War Veterans with Mild Traumatic Brain Injury â Flawed Perspectives

Thu, 16Â AprÂ 2009 00:00:00 -05:00

Researchers estimate that more than 300,000 U.S. veterans of the wars in Iraq and Afghanistan (20% of the 1.6 million) have sustained a mild traumatic brain injury (TBI), also known...

BOOK REVIEW:

Thu, 02Â AprÂ 2009 00:00:00 -05:00

As Torkel Klingberg points out in this book, the current milieu â in which there is an endless flow of cell phone messages and e-mails â is quite different from...

BOOK REVIEW:

Thu, 02Â AprÂ 2009 00:00:00 -05:00

The question âWhat matter?â is put to Alex the parrot by animal behaviorist Irene Pepperberg, who wants him to differentiate among wood, paper, and wool. Forget for a moment that...

BOOK REVIEW:

Thu, 19Â MarÂ 2009 00:00:00 -05:00

Since its origin, our earth has rotated approximately 1015 times, making light, and the lightâdark cycle, possibly the most potent selective forces acting on the evolution of all organisms on...

REVIEW ARTICLE: Moyamoya Disease and Moyamoya Syndrome

R. Michael Scott, M.D., and Edward R. Smith, M.D. — Thu, 19Â MarÂ 2009 00:00:00 -05:00

The moyamoya syndrome is a cerebrovascular condition that predisposes affected patients to stroke in association with progressive stenosis of the intracranial internal carotid arteries and their proximal branches. Reduced blood...

BOOK REVIEW:

Thu, 12Â MarÂ 2009 00:00:00 -05:00

In recent years, the public has been increasingly concerned about adverse events that have been attributed to vaccines. Although such safety concerns have existed since the days of Edward Jenner,...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 8-2009 â A 36-Year-Old Woman with Headache, Hypertension, and Seizure 2 Weeks Post Partum

Thu, 12Â MarÂ 2009 00:00:00 -05:00

Dr. W. Taylor Kimberly: A 36-year-old woman was admitted to the hospital because of headaches, hypertension, and seizures.

Nineteen days before the current admission, she delivered healthy twins at 35.6 weeks...

REVIEW ARTICLE: Primary Open-Angle Glaucoma

Thu, 12Â MarÂ 2009 00:00:00 -05:00

Glaucoma is a chronic, degenerative optic neuropathy that can be distinguished from most other forms of acquired optic neuropathy by the characteristic appearance of the optic nerve. 09031227.xml 09031227.xml 09031227.xml...

CLINICAL DECISIONS: Care of an Unresponsive Patient with a Poor Prognosis â Polling Results

Thu, 05Â MarÂ 2009 00:00:00 -05:00

In late January, we presented the case of an unresponsive, 56-year-old homeless patient who had a ruptured aneurysm, a high probability of cancer, and a best prognosis of severe disability...

IMAGES IN CLINICAL MEDICINE: Percussion Myotonia

Fabio A. Barroso, M.D. and Martin A. Nogues, M.D. — Thu, 05Â MarÂ 2009 00:00:00 -05:00

A 60-year-old woman came to the neuromuscular clinic because of a progressive gait difficulty that had started insidiously 2 years before presentation. She could walk and climb stairs unassisted but...

CLINICAL PRACTICE: Graves' Ophthalmopathy

Luigi Bartalena, M.D., and Maria Laura Tanda, M.D. — Thu, 05Â MarÂ 2009 00:00:00 -05:00

A 40-year-old woman who recently received a diagnosis of Graves' disease comes for a follow-up visit. She has been taking methimazole, at a dose of 10 mg daily, and is...

PERSPECTIVE: Serotonin Rising â The Bone, Brain, Bowel Connection

Thu, 05Â MarÂ 2009 00:00:00 -05:00

In a recent article, Yadav et al.1 elucidated the regulation of gut-produced serotonin by low-density lipoprotein receptorârelated protein 5 (Lrp5) and the deleterious effect of serotonin on bone mass. This...

CLINICAL PROBLEM-SOLVING: When Past Is Prologue

Thu, 05Â MarÂ 2009 00:00:00 -05:00

A 36-year-old man presented to the emergency department with a 2-week history of lower-extremity edema, progressive fatigue, and exertional dyspnea. The patient's medical history was notable for a fall from...

IMAGES IN CLINICAL MEDICINE: Pendular Nystagmus and Palatomyoclonus from Hypertrophic Olivary Degeneration

C.C. Tchoyoson Lim, M.D. and Su Ann Lim, M.D. — Thu, 26Â FebÂ 2009 00:00:00 -05:00

A 50-year-old woman with hypertension had an acute pontine hemorrhage, as seen on computed tomography (Panel A, arrow), which resulted in quadriplegia. Thirty months later, she reported having difficulty reading...

BOOK REVIEW:

Thu, 26Â FebÂ 2009 00:00:00 -05:00

It is estimated that more than 1.4 million people in the United States will receive a diagnosis of cancer in 2009 (excluding in situ carcinoma and nonmelanotic skin cancer), and...

BOOK REVIEW:

Thu, 26Â FebÂ 2009 00:00:00 -05:00

In the 1930s, Tracy Putnam and Houston Merritt codiscovered the antiepileptic properties of phenytoin (Dilantin). Their triumph is among the first translational partnerships between academia and pharmaceutical companies in which...

CORRESPONDENCE: Iodine Content of Prenatal Multivitamins in the United States

Angela M. Leung, M.D., Elizabeth N. Pearce, M.D., and Lewis E. Braverman, M.D. — Thu, 26Â FebÂ 2009 00:00:00 -05:00

To the Editor: Normal thyroid function in fetuses and breast-fed infants, which is dependent on sufficient maternal dietary intake of iodine, is crucial for normal neurocognitive development.1 Children of mothers...

IMAGES IN CLINICAL MEDICINE: Proliferative Diabetic Retinopathy

Pukhraj Rishi, M.S. and Pramod S. Bhende, M.S. — Thu, 26Â FebÂ 2009 00:00:00 -05:00

A 56-year-old man with diabetes presented with a several-month history of decreased vision in the left eye. There was no history of trauma, flashes of light, or floaters. Visual acuity...

ORIGINAL ARTICLE: Emergence of Ciprofloxacin-Resistant in North America

Thu, 26Â FebÂ 2009 00:00:00 -05:00

We report on three cases of meningococcal disease caused by ciprofloxacin-resistant Neisseria meningitidis, one in North Dakota and two in Minnesota. The cases were caused by the same serogroup B...

CORRESPONDENCE: Bezafibrate for an Inborn Mitochondrial Beta-Oxidation Defect

Thu, 19Â FebÂ 2009 00:00:00 -05:00

To the Editor: Carnitine palmitoyltransferase II (CPT2) deficiency is a rare autosomal recessive disorder of mitochondrial fatty acid oxidation. The most common form of this disorder is characterized by muscle...

EDITORIAL: Metabolic Enzymes as Oncogenes or Tumor Suppressors

Craig B. Thompson, M.D. — Thu, 19Â FebÂ 2009 00:00:00 -05:00

Whether mutations in metabolic pathways contribute to the pathogenesis of cancer is controversial.12 Cancer cells have long been noted to preferentially metabolize glucose through glycolysis, a discovery that has been...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 6-2009 â A 37-Year-Old Woman with Vertigo, Facial Weakness, and a Generalized Seizure

Didier Cros, M.D., R. Gilberto Gonzalez, M.D., Ph.D., and Eugene J. Mark, M.D. — Thu, 19Â FebÂ 2009 00:00:00 -05:00

Dr. Sang-Woo (Steve) Han (Neurology): A 37-year-old woman was seen in the emergency department of this hospital because of a seizure. Four months earlier, episodes of vertigo began to occur...

ORIGINAL ARTICLE: and Mutations in Gliomas

Thu, 19Â FebÂ 2009 00:00:00 -05:00

Background: A recent genomewide mutational analysis of glioblastomas (World Health Organization [WHO] grade IV glioma) revealed somatic mutations of the isocitrate dehydrogenase 1 gene (IDH1) in a fraction of such...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 5-2009 â A 47-Year-Old Woman with a Rash and Numbness and Pain in the Legs

Thu, 12Â FebÂ 2009 00:00:00 -05:00

Dr. John H. Stone: A 47-year-old woman was seen in the Rheumatology Clinic of this hospital because of numbness and pain in the legs and a rash.

The patient had been...

IMAGES IN CLINICAL MEDICINE: A Smooth, Shiny Tongue

Hyo Jin Lee, M.D. and Deog Yeon Jo, M.D. — Thu, 05Â FebÂ 2009 00:00:00 -05:00

A 77-year-old woman presented with an insidious onset of fatigue and a burning sensation of the tongue on swallowing food. Six years earlier, she had undergone a total gastrectomy for...

CORRESPONDENCE: Prevalence of Mitochondrial 1555AâG Mutation in Adults of European Descent

Thu, 05Â FebÂ 2009 00:00:00 -05:00

To the Editor: Sensorineural hearing loss is the most common type of sensory impairment worldwide.1 We have found that pathogenic mitochondrial DNA (mtDNA) mutations, such as the m.3243AâG mutation associated...

CORRESPONDENCE: Prevalence of Mitochondrial 1555AâG Mutation in European Children

Thu, 05Â FebÂ 2009 00:00:00 -05:00

To the Editor: Aminoglycoside antibiotics are used worldwide to treat gram-negative sepsis. Since these drugs are ototoxic and nephrotoxic, drug levels are closely monitored. However, their effect on patients with...

IMAGES IN CLINICAL MEDICINE: Traumatic Epidural Hematoma

Gary L. Gallia, M.D., Ph.D. and Michael H. Sobotta, M.B., B.S. — Thu, 05Â FebÂ 2009 00:00:00 -05:00

An 18-year-old intoxicated man was assaulted with a glass bottle on the left parietal region of his head and had a 5-minute loss of consciousness. The patient went home, but...

ORIGINAL ARTICLE: Mutations in in Autosomal Nonsyndromic Mental Retardation

Thu, 05Â FebÂ 2009 00:00:00 -05:00

Although autosomal forms of nonsyndromic mental retardation account for the majority of cases of mental retardation, the genes that are involved remain largely unknown. We sequenced the autosomal gene SYNGAP1,...

CLINICAL DECISIONS: Care of an Unresponsive Patient with a Poor Prognosis

Thu, 29Â JanÂ 2009 00:00:00 -05:00

A 56-year-old homeless man was found having a seizure and was transported to the hospital. He was found to have a subarachnoid hemorrhage and acute hydrocephalus. He underwent intubation, and...

BOOK REVIEW:

Thu, 08Â JanÂ 2009 00:00:00 -05:00

Asperger's Disorder is a fascinating compilation of scientific papers that concern a condition about which few people know anything. The book is a page turner â I could hardly put...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 1-2009 â A 57-Year-Old Man with Progressive Cognitive Decline

Thu, 08Â JanÂ 2009 00:00:00 -05:00

Dr. Matthew T. Bianchi (Neurology): A 57-year-old man was transferred to this hospital because of progressive cognitive decline. He had a history of recurrent malignant melanoma of the right conjunctiva...

IMAGES IN CLINICAL MEDICINE: Monocular Paralysis of Vertical Ductions after Facial Trauma

Hrachya Nersesyan, M.D., Ph.D. and Jorge C. Kattah, M.D. — Thu, 01Â JanÂ 2009 00:00:00 -05:00

A 44-year-old woman presented with painful diplopia while looking up or down after being struck in the left eye with a bare fist. She had no proptosis or ptosis, and...

CORRESPONDENCE: Persistent Fainting after Implantation of a âCurativeâ Pacemaker

Sazzli Kasim, M.R.C.P.I., Michael Hennessy, M.D., and James Crowley, M.D. — Thu, 01Â JanÂ 2009 00:00:00 -05:00

To the Editor: Syncope is a common and disabling problem, and its cause may be difficult to elucidate. A 64-year-old right-handed male taxi driver was referred to us for the...

CLINICAL PROBLEM-SOLVING: Keeping an Open Mind

Thu, 01Â JanÂ 2009 00:00:00 -05:00

A 67-year-old man presented with a 3-month history of fatigue and fever. He had undergone heart transplantation 6 years earlier for idiopathic cardiomyopathy. He reported no weight loss, night sweats,...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 40-2008 â A 26-Year-Old Man with Blurred Vision

Thu, 25Â DecÂ 2008 00:00:00 -05:00

Dr. Aaron Savar (Neuro-Ophthalmology): A 26-year-old man was seen in the Neuro-Ophthalmology Clinic of the Massachusetts Eye and Ear Infirmary (MEEI) because of blurred vision in the right eye.

Approximately 8...

BOOK REVIEW:

Thu, 18Â DecÂ 2008 00:00:00 -05:00

Ethical Issues in Neurology is firmly grounded in clinical practice and empirical data and is supported by conceptual and practical frameworks for action. James Bernat begins his book with theory...

BOOK REVIEW:

Thu, 18Â DecÂ 2008 00:00:00 -05:00

Dyslexia, an unexpected and unexplained difficulty in learning to read when cognitive function is otherwise normal, is a major disability. Much of the educational process in schools is based on...

REVIEW ARTICLE: Temporomandibular Disorders

Thu, 18Â DecÂ 2008 00:00:00 -05:00

The temporomandibular joint (TMJ) may be affected by inflammatory, traumatic, infectious, congenital, developmental, and neoplastic diseases, as seen in other joints. However, the most common affliction of the TMJ and...

BOOK REVIEW:

Thu, 11Â DecÂ 2008 00:00:00 -05:00

This book is about the great drug discoveries that were made by the legendary Albert Sjoerdsma and his team beginning in the 1950s. The first discoveries were made at the...

IMAGES IN CLINICAL MEDICINE: Central Pontine Myelinolysis

John D. Fleming, M.B., B.S. and Suresh Babu, M.B., B.S. — Thu, 04Â DecÂ 2008 00:00:00 -05:00

A 26-year-old man with a history of chronic alcohol abuse presented with dysarthria, lethargy, and horizontal nystagmus. Results of a clinical examination and blood tests were otherwise normal, including a...

CLINICAL PROBLEM-SOLVING: Taken Out of Context

Michael P. Thomas, M.D., and Andrew Wang, M.D. — Thu, 04Â DecÂ 2008 00:00:00 -05:00

A 25-year-old woman with a history of depression, mitral-valve prolapse, and migraine headaches presented to a hospital emergency department with a 3-day history of subjective fever, diffuse arthralgia, and severe...

EDITORIAL: The Genetics of Speech and Language Impairments

Karin Stromswold, M.D., Ph.D. — Thu, 27Â NovÂ 2008 00:00:00 -05:00

Without instruction, most children master the complexities of spoken language by the age of 6 or 7 years. About 5% of apparently healthy children, however, struggle to acquire basic competence...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 37-2008 â A 17-Year-Old Boy with a Pituitary Tumor and Skull Abnormalities

Thu, 27Â NovÂ 2008 00:00:00 -05:00

A 17-year-old boy was seen at this hospital because of a pituitary tumor and radiologic evidence of bony abnormalities in the skull. The patient was well until approximately 12 years...

CLINICAL PRACTICE: Primary Retinal Detachment

Donald J. D'Amico, M.D. — Thu, 27Â NovÂ 2008 00:00:00 -05:00

Rhegmatogenous retinal detachment, which refers to detachment due to round holes, tears, or breaks in the retina, is an important cause of visual symptoms and visual loss (Figure 1). The...

ORIGINAL ARTICLE: A Functional Genetic Link between Distinct Developmental Language Disorders

Thu, 27Â NovÂ 2008 00:00:00 -05:00

Background: Rare mutations affecting the FOXP2 transcription factor cause a monogenic speech and language disorder. We hypothesized that neural pathways downstream of FOXP2 influence more common phenotypes, such as specific...

CORRESPONDENCE: Restless Legs Syndrome and Spinal Anesthesia

Thu, 20Â NovÂ 2008 00:00:00 -05:00

To the Editor: The restless legs syndrome (RLS) is a common sensorimotor disorder of unknown cause affecting approximately 10% of the population.1 One uncontrolled study showed that spinal anesthesia caused...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 36-2008 â A 59-Year-Old Man with Chronic Daily Headache

Thu, 20Â NovÂ 2008 00:00:00 -05:00

Dr. Adam B. Cohen (Neurology): A 59-year-old man was admitted to the neurology service of this hospital because of chronic daily headache, fever, and myalgia.

Approximately 6 months earlier, the patient...

IMAGES IN CLINICAL MEDICINE: Cavernous Sinus Thrombosis

Ayaz Agayev, M.D. and Sabri Yilmaz, M.D. — Thu, 20Â NovÂ 2008 00:00:00 -05:00

A 34-year-old woman with a 3-year history of systemic lupus erythematosus was admitted to the hospital with sore throat and headache that had lasted for 3 weeks. She had been...

IMAGES IN CLINICAL MEDICINE: Ocular Myiasis

Michael A. Albert, Jr., M.D. and Jill R. Wells, M.D. — Thu, 13Â NovÂ 2008 00:00:00 -05:00

A 49-year-old man presented to the emergency room with a 2-day history of decreased vision in his left eye. He described his vision as âlooking through branches in a tree.â...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 35-2008 â A 65-Year-Old Man with Confusion and Memory Loss

Thu, 13Â NovÂ 2008 00:00:00 -05:00

Dr. Roy N. Alcalay (Neurology): A 65-year-old right-handed man was transferred to this hospital because of confusion and memory loss. He had been well until approximately 4 weeks before...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 35-2008 â A 65-Year-Old Man with Confusion and Memory Loss

Thu, 13Â NovÂ 2008 00:00:00 -05:00

Dr. Roy N. Alcalay (Neurology): A 65-year-old right-handed man was transferred to this hospital because of confusion and memory loss. He had been well until approximately 4 weeks before...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 35-2008 â A 65-Year-Old Man with Confusion and Memory Loss

Thu, 13Â NovÂ 2008 00:00:00 -05:00

Dr. Roy N. Alcalay (Neurology): A 65-year-old right-handed man was transferred to this hospital because of confusion and memory loss. He had been well until approximately 4 weeks before admission,...

IMAGES IN CLINICAL MEDICINE: Reversal of Traumatic Quadriplegia after Closed Reduction

John A. Cowan, Jr., M.D. and John E. McGillicuddy, M.D. — Thu, 13Â NovÂ 2008 00:00:00 -05:00

A 39-year-old woman was struck by a truck traveling at high speed. Cervical computed tomography showed a dislocation between the C5 and C6 vertebrae (Panel A, sagittal reconstruction). Physical examination...

ORIGINAL ARTICLE: Subthalamic Nucleus Stimulation in Severe ObsessiveâCompulsive Disorder

Thu, 13Â NovÂ 2008 00:00:00 -05:00

Background: Severe, refractory obsessiveâcompulsive disorder (OCD) is a disabling condition. Stimulation of the subthalamic nucleus, a procedure that is already validated for the treatment of movement disorders, has been proposed...

IMAGES IN CLINICAL MEDICINE: Binocular Proptosis in Orbital Lymphoma

Hielke J. Meulenbeld, M.D. and Wim Peters, M.D., Ph.D. — Thu, 06Â NovÂ 2008 00:00:00 -05:00

An 81-year-old man presented with a 7-month history of gradual, painless swelling and redness of both eyes. He reported blurred vision and weight loss of 10 kg (22 lb). The...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 34-2008 â A 58-Year-Old Woman with Neck Pain and Fever

Thu, 30Â OctÂ 2008 00:00:00 -05:00

Dr. Mary Pisculli (Infectious Disease): A 58-year-old woman was transferred to this hospital because of severe right-sided neck pain, fever, and abnormal findings on cervical magnetic resonance imaging (MRI).

Two to...

ORIGINAL ARTICLE: Aggressive vs. Conservative Phototherapy for Infants with Extremely Low Birth Weight

Thu, 30Â OctÂ 2008 00:00:00 -05:00

Background: It is unclear whether aggressive phototherapy to prevent neurotoxic effects of bilirubin benefits or harms infants with extremely low birth weight (1000 g or less).

Methods: We randomly assigned 1974...

IMAGES IN CLINICAL MEDICINE: Corneal Dendritic Ulcer from Herpes Simplex Virus Infection

Christopher C. Teng, M.D. — Thu, 23Â OctÂ 2008 00:00:00 -05:00

A 24-year-old man presented with a 2-day history of redness, photophobia, and blurry vision in the right eye. He reported no medical problems, similar previous episodes, fever, recent illnesses, or...

CORRESPONDENCE: Antiemetic Properties of the Antiepileptic Drug Levetiracetam

Thu, 23Â OctÂ 2008 00:00:00 -05:00

To the Editor: Chemotherapy-induced nausea and vomiting is an important problem in patients with brain tumors. Because of the high prevalence of seizures, most patients are given antiepileptic drugs. Motivated...

BOOK REVIEW:

Thu, 16Â OctÂ 2008 00:00:00 -05:00

Headache, one of the most common disorders, is a considerable burden to the individual and to society. The word âcomprehensiveâ in the title of this book may put off some...

EDITORIAL: Cytogenetic Technology â Genotype and Phenotype

David H. Ledbetter, Ph.D. — Thu, 16Â OctÂ 2008 00:00:00 -05:00

In this issue of the Journal, Mefford et al.1 describe an association between a microdeletion at 1q21.1 and a diverse range of impairments: mental retardation associated with microcephaly, cardiac abnormalities,...

ORIGINAL ARTICLE: Recurrent Rearrangements of Chromosome 1q21.1 and Variable Pediatric Phenotypes

Thu, 16Â OctÂ 2008 00:00:00 -05:00

Background: Duplications and deletions in the human genome can cause disease or predispose persons to disease. Advances in technologies to detect these changes allow for the routine identification of submicroscopic...

BOOK REVIEW:

Thu, 09Â OctÂ 2008 00:00:00 -05:00

Anyone who has had even one poor night of sleep will immediately recognize the substantial impact of sleep disturbance on quality of life. Sleep and Quality of Life in Clinical...

ORIGINAL ARTICLE: Toll-like Receptor 3 and Geographic Atrophy in Age-Related Macular Degeneration

Thu, 02Â OctÂ 2008 00:00:00 -05:00

Background: Age-related macular degeneration is the most common cause of irreversible visual impairment in the developed world. Advanced age-related macular degeneration consists of geographic atrophy and choroidal neovascularization. The specific...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Cell Therapies for Muscular Dystrophy

Helen M. Blau, Ph.D. — Thu, 25Â SepÂ 2008 00:00:00 -05:00

Duchenne's muscular dystrophy is a devastating, progressive, X-linked muscle-wasting disease. It is the most common form of muscular dystrophy, affecting 1 in 3500 boys. With an onset in early childhood,...

BOOK REVIEW:

Thu, 18Â SepÂ 2008 00:00:00 -05:00

The earliest symptoms of autism spectrum disorders often appear before a child's second birthday, but most children with autism are not diagnosed until they are in preschool or elementary school....

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Turning Thought into Action

Leigh R. Hochberg, M.D., Ph.D. — Thu, 11Â SepÂ 2008 00:00:00 -05:00

Patients with spinal cord injury, subcortical stroke, neuromuscular diseases (including amyotrophic lateral sclerosis), and limb amputation have at least two characteristics in common: a brain that wants to direct movement...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 28-2008 â An 8-Day-Old Infant with Congenital Deafness, Lethargy, and Hypothermia

Thu, 11Â SepÂ 2008 00:00:00 -05:00

Dr. Sarah M. Barnett (Neurology): An 8-day-old boy was admitted to the neonatal intensive care unit of this hospital because of weakness and lethargy.

The patient was born after a full-term...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Targeting Intraocular Neovascularization and Edema â One Drop at a Time

Lloyd Paul Aiello, M.D., Ph.D. — Thu, 28Â AugÂ 2008 00:00:00 -05:00

Barring death, blindness is one of the most feared complications of human disease. In developed countries worldwide, the vast majority of visual loss results from pathologic neovascularization of the choroid...

EDITORIAL: Antenatal Magnesium Sulfate for Neuroprotection before Preterm Birth?

Fiona J. Stanley, M.D., and Caroline Crowther, F.R.A.N.Z.C.O.G. — Thu, 28Â AugÂ 2008 00:00:00 -05:00

Preterm infants are at increased risk for serious, lifelong neurologic abnormalities such as cerebral palsy.12 As the survival of preterm infants has improved with advances in perinatal care,2 the occurrence...

ORIGINAL ARTICLE: Brain-Derived Neurotrophic Factor and Obesity in the WAGR Syndrome

Thu, 28Â AugÂ 2008 00:00:00 -05:00

Background: Brain-derived neurotrophic factor (BDNF) has been found to be important in energy homeostasis in animal models, but little is known about its role in energy balance in humans. Heterozygous,...

ORIGINAL ARTICLE: A Randomized, Controlled Trial of Magnesium Sulfate for the Prevention of Cerebral Palsy

Thu, 28Â AugÂ 2008 00:00:00 -05:00

Background: Research suggests that fetal exposure to magnesium sulfate before preterm birth might reduce the risk of cerebral palsy.

Methods: In this multicenter, placebo-controlled, double-blind trial, we randomly assigned women at...

EDITORIAL: Pharmacogenomics and Drug Toxicity

Yusuke Nakamura, M.D., Ph.D. — Thu, 21Â AugÂ 2008 00:00:00 -05:00

In the United States alone, it is estimated that adverse drug reactions affect nearly 2 million patients and kill about 100,000 people each year.1 Adverse drug reactions are often classified...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 26-2008 â A 26-Year-Old Woman with Headache and Behavioral Changes

Thomas D. Sabin, M.D., Jeffrey A. Jednacz, M.D., and Paul N. Staats, M.D. — Thu, 21Â AugÂ 2008 00:00:00 -05:00

Dr. Thomas J. Cummings, Jr. (Psychiatry): A 26-year-old woman was admitted to this hospital because of headache, behavioral changes, abnormal movements, and inability to communicate. The patient had been well,...

CLINICAL PRACTICE: Idiopathic Sudden Sensorineural Hearing Loss

Steven D. Rauch, M.D. — Thu, 21Â AugÂ 2008 00:00:00 -05:00

A healthy 58-year-old woman answers the telephone and realizes that her hearing is diminished on the left side. She notices aural fullness and loud tinnitus in the affected ear. Later...

ORIGINAL ARTICLE: Variants and Statin-Induced Myopathy â A Genomewide Study

The SEARCH Collaborative Group — Thu, 21Â AugÂ 2008 00:00:00 -05:00

Background: Lowering low-density lipoprotein cholesterol with statin therapy results in substantial reductions in cardiovascular events, and larger reductions in cholesterol may produce larger benefits. In rare cases, myopathy occurs in...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 25-2008 â A 43-Year-Old Man with Fatigue and Lesions in the Pituitary and Cerebellum

John A. Mills, M.D., R. Gilberto Gonzalez, M.D., Ph.D., and Ronald Jaffe, M.D. — Thu, 14Â AugÂ 2008 00:00:00 -05:00

Dr. Flavia V. Castelino (Rheumatology, Allergy, and Immunology): A 43-year-old man was seen in the neuro-oncology clinic of this hospital because of fatigue and lesions in the pituitary and cerebellum...

IMAGES IN CLINICAL MEDICINE: Horizontal Pendular Nystagmus in a Patient with Ocular Albinism

Ariel D. Teitel, M.D. and Josh Rubin, M.D. — Thu, 31Â JulÂ 2008 00:00:00 -05:00

A 32-year-old Hispanic woman with ocular albinism presented with a congenital horizontal pendular nystagmus (see video). Other than being fair-skinned, the patient had no cutaneous manifestations of albinism, and pigmentation...

IMAGES IN CLINICAL MEDICINE: Complex Regional Pain Syndrome Type 1

Oliver Guttmann, M.B., B.S. and Victoria Wykes, M.B., B.S., Ph.D. — Thu, 31Â JulÂ 2008 00:00:00 -05:00

An otherwise healthy and functional 35-year-old man had an undisplaced fracture of the right fifth metacarpal neck after a fall. He presented 21 months later with pain, extensive swelling (Panels...

REVIEW ARTICLE: Malignant Gliomas in Adults

Patrick Y. Wen, M.D., and Santosh Kesari, M.D., Ph.D. — Thu, 31Â JulÂ 2008 00:00:00 -05:00

Malignant gliomas account for approximately 70% of the 22,500 new cases of malignant primary brain tumors that are diagnosed in adults in the United States each year.123 Although relatively uncommon,...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 22-2008 â A 52-Year-Old Woman with Fever and Confusion

Thu, 17Â JulÂ 2008 00:00:00 -05:00

Dr. Marylyn M. Addo (Infectious Disease): A 52-year-old woman was admitted to this hospital in late summer because of fever and confusion. The patient had been well until 9 days...

CLINICAL PRACTICE: Initial Management of Epilepsy

Jacqueline A. French, M.D., and Timothy A. Pedley, M.D. — Thu, 10Â JulÂ 2008 00:00:00 -05:00

A 29-year-old woman presents for evaluation. The previous evening, her husband, who was in the next room, heard unusual sounds and found her lying on the bed looking dazed. She...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 20-2008 â A 57-Year-Old Woman with Abdominal Pain and Weakness after Gastric Bypass Surgery

Thu, 26Â JunÂ 2008 00:00:00 -05:00

Dr. Allyson K. Bloom (Medicine): A 57-year-old woman was admitted to this hospital because of abdominal pain and weakness. One month earlier, a laparoscopic Roux-en-Y gastric bypass and cholecystectomy were...

IMAGES IN CLINICAL MEDICINE: Central Hemiretinal Arterial Occlusion

Kenneth C. Mathys, M.D. and Seema Garg, M.D., Ph.D. — Thu, 19Â JunÂ 2008 00:00:00 -05:00

A 65-year-old woman with a history of hypertension and cigarette smoking presented with complete loss of vision in the inferior field of the left eye, which she had noticed an...

ORIGINAL ARTICLE: Electrocardiographic Abnormalities and Sudden Death in Myotonic Dystrophy Type 1

Thu, 19Â JunÂ 2008 00:00:00 -05:00

Background: Sudden death can occur as a consequence of cardiac-conduction abnormalities in the neuromuscular disease myotonic dystrophy type 1. The determinants of the risk of sudden death remain imprecise.

Methods: We...

EDITORIAL: Neuroblastoma â Linking a Common Allele to a Rare Disease

Brian H. Kushner, M.D., and Nai-Kong V. Cheung, M.D., Ph.D. — Thu, 12Â JunÂ 2008 00:00:00 -05:00

Neuroblastoma has a notorious reputation among solid tumors of childhood because of its frequently massive and widespread tumor burden. Yet stage for stage, this embryonal neoplasm of the sympathetic nervous...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 18-2008 â A 68-Year-Old Man with Headache and Visual Changes after Liver Transplantation

Thu, 12Â JunÂ 2008 00:00:00 -05:00

A 68-year-old man was admitted to the hospital because of headache and loss of vision in the right eye. Orthotopic allogeneic liver transplantation had been performed 3 1/2 months earlier...

REVIEW ARTICLE: Age-Related Macular Degeneration

Rama D. Jager, M.D., William F. Mieler, M.D., and Joan W. Miller, M.D. — Thu, 12Â JunÂ 2008 00:00:00 -05:00

Age-related macular degeneration is the leading cause of irreversible blindness in people 50 years of age or older in the developed world.12 More than 8 million Americans have age-related macular...

ORIGINAL ARTICLE: Chromosome 6p22 Locus Associated with Clinically Aggressive Neuroblastoma

Thu, 12Â JunÂ 2008 00:00:00 -05:00

Background: Neuroblastoma is a malignant condition of the developing sympathetic nervous system that most commonly affects young children and is often lethal. Its cause is not known.

Methods: We performed a...

IMAGES IN CLINICAL MEDICINE: Ethmoid Mucocele Causing Visual Impairment

Jae Yong Lee, M.D., Ph.D. — Thu, 05Â JunÂ 2008 00:00:00 -05:00

A 74-year-old man was referred because of left exophthalmos and outward displacement of the orbit (Panel A). His symptoms had progressed slowly over a 4-year period but had worsened rapidly...

ORIGINAL ARTICLE: Hypothermia Therapy after Traumatic Brain Injury in Children

Thu, 05Â JunÂ 2008 00:00:00 -05:00

Background: Hypothermia therapy improves survival and the neurologic outcome in animal models of traumatic brain injury. However, the effect of hypothermia therapy on the neurologic outcome and mortality among children...

EDITORIAL: Preliminary Results of Gene Therapy for Retinal Degeneration

Joan W. Miller, M.D. — Thu, 22Â MayÂ 2008 00:00:00 -05:00

In this issue of the Journal, two groups of investigators â Bainbridge et al.1 and Maguire et al.2 â describe the first results of separate clinical trials investigating the short-term...

IMAGES IN CLINICAL MEDICINE: Ocular Trauma Due to a Water-Bottle Cap

Shiyoung Roh, M.D. and Mark E. Patron, M.D. — Thu, 22Â MayÂ 2008 00:00:00 -05:00

A 14-year-old girl presented with a traumatic injury to the right eye from a water-bottle cap, caused by the increasingly popular adolescent activity of twisting and crushing an empty plastic...

ORIGINAL ARTICLE: Safety and Efficacy of Gene Transfer for Leber's Congenital Amaurosis

Thu, 22Â MayÂ 2008 00:00:00 -05:00

Leber's congenital amaurosis (LCA) is a group of inherited blinding diseases with onset during childhood. One form of the disease, LCA2, is caused by mutations in the retinal pigment epitheliumâspecific...

ORIGINAL ARTICLE: Effect of Gene Therapy on Visual Function in Leber's Congenital Amaurosis

Thu, 22Â MayÂ 2008 00:00:00 -05:00

Early-onset, severe retinal dystrophy caused by mutations in the gene encoding retinal pigment epitheliumâspecific 65-kD protein (RPE65) is associated with poor vision at birth and complete loss of vision in...

CLINICAL DECISIONS: Management of Carotid Stenosis â Polling Results

Thu, 15Â MayÂ 2008 00:00:00 -05:00

In early April, we presented the case of a 67-year-old man with asymptomatic carotid disease in Clinical Decisions,1 an interactive feature designed to assess how readers would manage a clinical...

EDITORIAL: Intracerebral Hemorrhage â Improving Outcome by Reducing Volume?

Stanley Tuhrim, M.D. — Thu, 15Â MayÂ 2008 00:00:00 -05:00

Intracerebral hemorrhage accounts for 10 to 15% of all strokes. It is the type of stroke with the highest mortality, with a 1-year survival rate of less than 50%.1 Most...

ORIGINAL ARTICLE: Efficacy and Safety of Recombinant Activated Factor VII for Acute Intracerebral Hemorrhage

Thu, 15Â MayÂ 2008 00:00:00 -05:00

Background: Intracerebral hemorrhage is the least treatable form of stroke. We performed this phase 3 trial to confirm a previous study in which recombinant activated factor VII (rFVIIa) reduced growth...

PERSPECTIVE: Vaccines and Autism Revisited â The Hannah Poling Case

Thu, 15Â MayÂ 2008 00:00:00 -05:00

On April 11, 2008, the National Vaccine Advisory Committee took an unusual step: in the name of transparency, trust, and collaboration, it asked members of the public to help set...

IMAGES IN CLINICAL MEDICINE: Changes in the Brain Stem and Fundus in Malignant Hypertension

Michael S. Lee, M.D. and Brian J. Tienor, B.S. — Thu, 01Â MayÂ 2008 00:00:00 -05:00

A 53-year-old man presented with a 4-week history of bilateral retrobulbar headache and blurred vision. His blood pressure was 220/135 mm Hg; his neurologic examination was unremarkable. He had no...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 12-2008 â A Newborn Infant with Intermittent Apnea and Seizures

Thu, 17Â AprÂ 2008 00:00:00 -05:00

A 1-day-old female infant was admitted to the neonatal intensive care unit of this hospital because of intermittent apnea and cyanosis. The day before admission, the patient was born at...

IMAGES IN CLINICAL MEDICINE: Cerebral Embolism of Probable Aortic Origin

Ronald E. Oppenheim, M.D. and Gary J. Felsberg, M.D. — Thu, 10Â AprÂ 2008 00:00:00 -05:00

A 70-year-old woman was hospitalized for intermittent, reversible cerebral ischemia causing weakness of the right side for several days, before resolving. A computed tomographic (CT) scan showed no intracranial abnormalities....

CLINICAL DECISIONS: Management of Carotid Stenosis

Thu, 10Â AprÂ 2008 00:00:00 -05:00

A 67-year-old man with a history of hypertension and hyperlipidemia is seen for a routine examination. His medications include hydrochlorothiazide (25 mg daily), simvastatin (20 mg daily), and aspirin (81...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 11-2008 â A 45-Year-Old Man with Changes in Mental Status after Liver Transplantation

Thu, 10Â AprÂ 2008 00:00:00 -05:00

A 45-year-old man was admitted to this hospital for orthotopic liver transplantation. The patient had been well until the age of 18 years, when a diagnosis of non-A, non-B hepatitis...

IMAGES IN CLINICAL MEDICINE: Spontaneous Otogenic Pneumocephalus

Roberto A. Villa, M.D. and Abelardo Capdevila, M.D. — Thu, 20Â MarÂ 2008 00:00:00 -05:00

A healthy 54-year-old woman presented with progressive abnormal acoustic sensations, aphasia, and visual-field disturbances. She reported no head trauma or recent infection, such as otitis media. An initial cranial radiograph...

IMAGES IN CLINICAL MEDICINE: Retinal Arteriolar Cholesterol Emboli

Michael Colucciello, M.D. — Thu, 21Â FebÂ 2008 00:00:00 -05:00

A 59-year-old man with a medical history of hypertension, hyperlipidemia, and coronary artery disease presented with transient, painless visual obscuration in the left eye, and he was referred for retinal...

EDITORIAL: A Hot Spot of Genetic Instability in Autism

Evan E. Eichler, Ph.D., and Andrew W. Zimmerman, M.D. — Thu, 14Â FebÂ 2008 00:00:00 -05:00

Sixty-five years after Leo Kanner first described autism,1 we are beginning to move beyond description of this clinically heterogeneous neurobehavioral syndrome toward a deeper understanding of its biologic complexity. In...

ORIGINAL ARTICLE: Association between Microdeletion and Microduplication at 16p11.2 and Autism

Thu, 14Â FebÂ 2008 00:00:00 -05:00

Background: Autism spectrum disorder is a heritable developmental disorder in which chromosomal abnormalities are thought to play a role.

Methods: As a first component of a genomewide association study of families...

CLINICAL PRACTICE: Neurogenic Orthostatic Hypotension

Roy Freeman, M.B., Ch.B. — Thu, 07Â FebÂ 2008 00:00:00 -05:00

A 65-year-old man reports a 6-month history of dizziness, light-headedness, weakness, and fatigue while upright. He takes no medication and has no personal or family history of neurologic disease. On...

ORIGINAL ARTICLE: Neonatal Diagnosis and Treatment of Menkes Disease

Thu, 07Â FebÂ 2008 00:00:00 -05:00

Background: Menkes disease is a fatal neurodegenerative disorder of infancy caused by diverse mutations in a copper-transport gene, ATP7A. Early treatment with copper injections may prevent death and illness, but...

EDITORIAL: Disentangling Mild Traumatic Brain Injury and Stress Reactions

Richard A. Bryant, Ph.D. — Thu, 31Â JanÂ 2008 00:00:00 -05:00

The study by Hoge and colleagues in this issue of the Journal provides an important profile of the sequelae of mild traumatic brain injury in military personnel after combat.1 The...

ORIGINAL ARTICLE: Mild Traumatic Brain Injury in U.S. Soldiers Returning from Iraq

Thu, 31Â JanÂ 2008 00:00:00 -05:00

Background: An important medical concern of the Iraq war is the potential long-term effect of mild traumatic brain injury, or concussion, particularly from blast explosions. However, the epidemiology of combat-related...

IMAGES IN CLINICAL MEDICINE: Penetrating Orbital Trauma

Harsha S. Reddy, M.D. and Eli Chang, M.D. — Thu, 17Â JanÂ 2008 00:00:00 -05:00

A 15-year-old boy presented with an inability to open his left eye after being assaulted with a pencil. A small laceration was noted on the left eyebrow (Panel A). An...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 2-2008 â A 38-Year-Old Woman with Postpartum Visual Loss, Shortness of Breath, and Renal Failure

Thu, 17Â JanÂ 2008 00:00:00 -05:00

Dr. Esperance A. Schaefer (Medicine): A 38-year-old woman was admitted to the hospital, because of blurred vision and shortness of breath, 2 weeks after a cesarean section had been performed...

EDITORIAL: Efficacy of Sirolimus in Treating Tuberous Sclerosis and Lymphangioleiomyomatosis

Elahna Paul, M.D., Ph.D., and Elizabeth Thiele, M.D., Ph.D. — Thu, 10Â JanÂ 2008 00:00:00 -05:00

Owing to their immunosuppressive and antiproliferative effects, sirolimus (also called rapamycin) and related drugs are being evaluated as part of many transplant immunosuppresion regimens, as well as for a plethora...

CLINICAL PRACTICE: Long-QT Syndrome

Dan M. Roden, M.D. — Thu, 10Â JanÂ 2008 00:00:00 -05:00

After the sudden death of a 13-year-old girl while she was playing basketball, her family comes to the clinic for medical evaluation (Figure 1). Her parents' resting electrocardiograms (ECGs) are...

ORIGINAL ARTICLE: Sirolimus for Angiomyolipoma in Tuberous Sclerosis Complex or Lymphangioleiomyomatosis

Thu, 10Â JanÂ 2008 00:00:00 -05:00

Background: Angiomyolipomas in patients with the tuberous sclerosis complex or sporadic lymphangioleiomyomatosis are associated with mutations in tuberous sclerosis genes resulting in constitutive activation of the mammalian target of rapamycin...

IMAGES IN CLINICAL MEDICINE: Medical Mystery: Cloud Surrounding the Optic Disks

Sandeep Randhawa, M.D. and Andrew G. Lee, M.D. — Thu, 03Â JanÂ 2008 00:00:00 -05:00

A 42-year-old man presented for a routine eye examination. His best corrected visual acuity was 20/20 in both eyes, and examination of the anterior segment of each eye showed no...

PERSPECTIVE: Seeking God in the Brain â Efforts to Localize Higher Brain Functions

Thu, 03Â JanÂ 2008 00:00:00 -05:00

Neuroscientists have long eschewed global questions about brain function, and books reviewing the current state of neuroscience usually allocate only a small section to âhigher functions.â But with the advent...

ORIGINAL ARTICLE: Functional Outcome after Language Mapping for Glioma Resection

Nader Sanai, M.D., Zaman Mirzadeh, Ph.D., and Mitchel S. Berger, M.D. — Thu, 03Â JanÂ 2008 00:00:00 -05:00

Background: Language sites in the cortex of the brain vary among patients. Language mapping while the patient is awake is an intraoperative technique designed to minimize language deficits associated with...

EDITORIAL: Skipping toward Personalized Molecular Medicine

Eric P. Hoffman, Ph.D. — Thu, 27Â DecÂ 2007 00:00:00 -05:00

âPersonalized molecular medicine.â As with other catchy terms for big ideas, such as âreversing global warmingâ and ârenewable energy,â the concept of personalized molecular medicine is certainly important, but the...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 40-2007 â A 38-Year-Old Man with Weakness in the Hands

William J. Triggs, M.D., and Didier Cros, M.D. — Thu, 27Â DecÂ 2007 00:00:00 -05:00

A 38-year-old, right-handed man was seen in the neuromuscular unit of this hospital because of weakness of the hands. He first noted shaking of his hands, the right more than...

ORIGINAL ARTICLE: Mutations and Hereditary Angiopathy, Nephropathy, Aneurysms, and Muscle Cramps

Thu, 27Â DecÂ 2007 00:00:00 -05:00

Background: COL4A3, COL4A4, and COL4A5 are the only collagen genes that have been implicated in inherited nephropathies in humans. However, the causative genes for a number of hereditary multicystic kidney...

ORIGINAL ARTICLE: Local Dystrophin Restoration with Antisense Oligonucleotide PRO051

Thu, 27Â DecÂ 2007 00:00:00 -05:00

Background: Duchenne's muscular dystrophy is associated with severe, progressive muscle weakness and typically leads to death between the ages of 20 and 35 years. By inducing specific exon skipping during...

IMAGES IN CLINICAL MEDICINE: Dissecting Aneurysm of the Posterior Cerebral Artery

Dimitri Renard, M.D. and Didier Milhaud, M.D. — Thu, 13Â DecÂ 2007 00:00:00 -05:00

A 43-year-old man presented with acute-onset hemianesthesia involving the right arm, the right leg, and the right side of the face. Six weeks earlier, he had a moderate occipital headache...

EDITORIAL: Corticosteroids for Acute Bacterial Meningitis

Brian M. Greenwood, M.D. — Thu, 13Â DecÂ 2007 00:00:00 -05:00

Death and long-term disabilities are common outcomes of acute bacterial meningitis, especially in developing countries, even when highly effective antibiotic therapy is given. Therefore, improvement in the outcomes of acute...

IMAGES IN CLINICAL MEDICINE: Occlusion and Reperfusion of the Middle Cerebral Artery

Ronan Ryan, A.F.R.C.S.I. and David P. Brophy, F.F.R.R.C.S.I. — Thu, 13Â DecÂ 2007 00:00:00 -05:00

A 65-year-old woman with chronic atrial fibrillation was admitted for an elective exchange of an implanted defibrillator for idiopathic dilated cardiomyopathy. To facilitate this procedure, warfarin was withheld for 5...

ORIGINAL ARTICLE: Corticosteroids for Bacterial Meningitis in Adults in Sub-Saharan Africa

Thu, 13Â DecÂ 2007 00:00:00 -05:00

Background: In sub-Saharan Africa, bacterial meningitis is common and is associated with a high mortality. Adjuvant therapy with corticosteroids reduces mortality among adults in the developed world, but it has...

ORIGINAL ARTICLE: Dexamethasone in Vietnamese Adolescents and Adults with Bacterial Meningitis

Thu, 13Â DecÂ 2007 00:00:00 -05:00

Background: It is uncertain whether all adults with bacterial meningitis benefit from treatment with adjunctive dexamethasone.

Methods: We conducted a randomized, double-blind, placebo-controlled trial of dexamethasone in 435 patients over the...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Molecules That Mediate Mood

Michael E. Thase, M.D. — Thu, 06Â DecÂ 2007 00:00:00 -05:00

Major depressive disorder is now recognized as one of the world's greatest public health problems: it is common, typically follows a relapsing or recurrent course, disrupts the performance of social...

CLINICAL THERAPEUTICS: Cochlear Implants for Children with Severe-to-Profound Hearing Loss

Blake C. Papsin, M.D., and Karen A. Gordon, Ph.D. — Thu, 06Â DecÂ 2007 00:00:00 -05:00

A 4-month-old baby girl whose parents and 6-year-old brother have severe-to-profound sensorineural hearing loss is referred for consideration for cochlear implantation. Each of the baby's parents had previously received a...

IMAGES IN CLINICAL MEDICINE: Cerebral Tuberculoma

Juan Carlos CataÃ±o, M.D. — Thu, 22Â NovÂ 2007 00:00:00 -05:00

A 29-year-old homeless man was admitted to the hospital after being stabbed in the chest. A hemothorax was diagnosed, and a chest tube was placed. One day after admission, he...

IMAGES IN CLINICAL MEDICINE: Harlequin's Darker Side

Martin E. Duddy, M.R.C.P. and Mark R. Baker, M.R.C.P. — Thu, 15Â NovÂ 2007 00:00:00 -05:00

A 39-year-old healthy, female aerobics instructor presented with a 6-week history of flushing on the left side of her face, neck, and upper trunk after 40 minutes of vigorous exercise,...

EDITORIAL: Congenital Heart Disease and Brain Injury

Michael V. Johnston, M.D. — Thu, 08Â NovÂ 2007 00:00:00 -05:00

During the past 25 years, remarkable progress has been made in the surgical treatment and survival of infants with congenital heart disease.1 Recent data have shown that in-hospital death rates...

EDITORIAL: On the Caffeination of Prematurity

David K. Stevenson, M.D. — Thu, 08Â NovÂ 2007 00:00:00 -05:00

It was recognized more than three decades ago1 that methylxanthines can reduce the frequency of apneic episodes in premature infants. Since that time, methylxanthines have become part of the routine...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 34-2007 â A 77-Year-Old Man with Ear Pain, Difficulty Speaking, and Altered Mental Status

Thu, 08Â NovÂ 2007 00:00:00 -05:00

A 77-year-old right-handed man was admitted to the hospital because of the recent onset of pain in the ear, difficulty speaking, and altered mental status. The patient had been well...

ORIGINAL ARTICLE: Abnormal Brain Development in Newborns with Congenital Heart Disease

Thu, 08Â NovÂ 2007 00:00:00 -05:00

Background: Congenital heart disease in newborns is associated with global impairment in development. We characterized brain metabolism and microstructure, as measures of brain maturation, in newborns with congenital heart disease...

ORIGINAL ARTICLE: Long-Term Effects of Caffeine Therapy for Apnea of Prematurity

Thu, 08Â NovÂ 2007 00:00:00 -05:00

Background: Methylxanthine therapy is commonly used for apnea of prematurity but in the absence of adequate data on its efficacy and safety. It is uncertain whether methylxanthines have long-term effects...

ORIGINAL ARTICLE: Visualizing Out-of-Body Experience in the Brain

Thu, 01Â NovÂ 2007 00:00:00 -05:00

An out-of-body experience was repeatedly elicited during stimulation of the posterior part of the superior temporal gyrus on the right side in a patient in whom electrodes had been implanted...

ORIGINAL ARTICLE: Incidental Findings on Brain MRI in the General Population

Thu, 01Â NovÂ 2007 00:00:00 -05:00

Background: Magnetic resonance imaging (MRI) of the brain is increasingly used both in research and in clinical medicine, and scanner hardware and MRI sequences are continually being improved. These advances...

IMAGES IN CLINICAL MEDICINE: Magnetic Resonance Imaging of Retinal Detachment

Ingo NÃ¶lte, M.D. and Marc A. Brockmann, M.D. — Thu, 25Â OctÂ 2007 00:00:00 -05:00

An 80-year-old woman with a myopia of â19.0 diopters and a history of right-sided retinal detachment underwent a diagnostic workup for a decrease of vision in her right eye. The...

EDITORIAL: Bell's Palsy â Is Glucocorticoid Treatment Enough?

Donald H. Gilden, M.D., and Kenneth L. Tyler, M.D. — Thu, 18Â OctÂ 2007 00:00:00 -05:00

Approximately a third of cases of acute peripheral facial weakness are caused by trauma, diabetes mellitus, hypertension, eclampsia, the Ramsay Hunt syndrome (facial palsy with zoster oticus caused by varicellaâzoster...

ORIGINAL ARTICLE: Early Treatment with Prednisolone or Acyclovir in Bell's Palsy

Thu, 18Â OctÂ 2007 00:00:00 -05:00

Background: Corticosteroids and antiviral agents are widely used to treat the early stages of idiopathic facial paralysis (i.e., Bell's palsy), but their effectiveness is uncertain.

Methods: We conducted a double-blind, placebo-controlled,...

EDITORIAL: Treatment of Neuropsychiatric Symptoms in Patients with Dementia

Kristine Yaffe, M.D. — Thu, 04Â OctÂ 2007 00:00:00 -05:00

Although cognitive deficits are the clinical hallmark of dementia syndromes, noncognitive symptoms are common and can dominate the disease course. These include a range of neuropsychiatric symptoms, such as agitation,...

REVIEW ARTICLE: A Critical Appraisal of âChronic Lyme Diseaseâ

Thu, 04Â OctÂ 2007 00:00:00 -05:00

07100428.xml 07100428.xml 07100428.xml 07100428.xml Lyme disease, the most common tick-borne infection in the northern hemisphere, is a serious public health problem. In North America, it is caused exclusively by...

ORIGINAL ARTICLE: Donepezil for the Treatment of Agitation in Alzheimer's Disease

Thu, 04Â OctÂ 2007 00:00:00 -05:00

Background: Agitation is a common and distressing symptom in patients with Alzheimer's disease. Cholinesterase inhibitors improve cognitive outcomes in such patients, but the benefits of these drugs for behavioral disturbances...

ORIGINAL ARTICLE: Early Thimerosal Exposure and Neuropsychological Outcomes at 7 to 10 Years

Thu, 27Â SepÂ 2007 00:00:00 -05:00

Background: It has been hypothesized that early exposure to thimerosal, a mercury-containing preservative used in vaccines and immune globulin preparations, is associated with neuropsychological deficits in children.

Methods: We enrolled 1047...

PERSPECTIVE: Cases in Vaccine Court â Legal Battles over Vaccines and Autism

Thu, 27Â SepÂ 2007 00:00:00 -05:00

Do childhood vaccines cause autism? This scientific question has now become a legal one â perhaps inevitable in our society. Some families with autistic children are pursuing legal channels in...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: On Prions, Proteasomes, and Mad Cows

Alfred L. Goldberg, Ph.D. — Thu, 13Â SepÂ 2007 00:00:00 -05:00

All cells have the capacity to selectively degrade misfolded intracellular proteins, which, if they accumulated, could interfere with normal function and could be toxic. Such proteins may arise by mutation,...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 28-2007 â A 68-Year-Old Man with Syncope

Thu, 13Â SepÂ 2007 00:00:00 -05:00

A 68-year-old man was admitted to this hospital because of a syncopal episode, followed by hemiparesis and altered mental status.

The patient was in his usual state of health until the...

IMAGES IN CLINICAL MEDICINE: Pneumocephalus Associated with Pneumosinus Dilatans Frontalis

Neil Bhattacharyya, M.D. and Robert M. Friedlander, M.D. — Thu, 13Â SepÂ 2007 00:00:00 -05:00

An 87-year-old woman presented to the emergency department with an acute-onset severe global headache of 36 hours' duration, with greater occipital involvement than frontal involvement. She reported no recent trauma,...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: A Tale about Tau

Karen H. Ashe, M.D., Ph.D. — Thu, 30Â AugÂ 2007 00:00:00 -05:00

During the next few decades, millions of baby boomers will enter their 70s, 80s, and 90s, quadrupling the number of patients with Alzheimer's disease, the leading cause of dementia in...

ORIGINAL ARTICLE: Saline or Albumin for Fluid Resuscitation in Patients with Traumatic Brain Injury

The SAFE Study Investigators — Thu, 30Â AugÂ 2007 00:00:00 -05:00

Background: The Saline versus Albumin Fluid Evaluation study suggested that patients with traumatic brain injury resuscitated with albumin had a higher mortality rate than those resuscitated with saline. We conducted...

EDITORIAL: Understanding the Causes of Amyotrophic Lateral Sclerosis

Richard W. Orrell, M.D. — Thu, 23Â AugÂ 2007 00:00:00 -05:00

Amyotrophic lateral sclerosis (ALS) is a human disease resulting from the degeneration of motor neurons in the brain, spinal cord, and peripheral nervous system. The resultant clinical features include weakness...

ORIGINAL ARTICLE: Whole-Genome Analysis of Sporadic Amyotrophic Lateral Sclerosis

Thu, 23Â AugÂ 2007 00:00:00 -05:00

Background: Approximately 90% of persons with amyotrophic lateral sclerosis (ALS) have the sporadic form, which may be caused by the interaction of multiple environmental factors and previously unknown genes.

Methods: We...

EDITORIAL: Periodic Limb Movements in Sleep â Endophenotype for Restless Legs Syndrome?

John W. Winkelman, M.D., Ph.D. — Thu, 16Â AugÂ 2007 00:00:00 -05:00

Today, 350 years after its first description, the restless legs syndrome (RLS) is strangely controversial. Experts and patients alike describe RLS both as a miserably impairing disorder and as âthe...

ORIGINAL ARTICLE: Prophylactic Cranial Irradiation in Extensive Small-Cell Lung Cancer

Thu, 16Â AugÂ 2007 00:00:00 -05:00

Background: We conducted a randomized trial of prophylactic cranial irradiation in patients with extensive small-cell lung cancer who had had a response to chemotherapy.

Methods: Patients between the ages of 18...

ORIGINAL ARTICLE: A Genetic Risk Factor for Periodic Limb Movements in Sleep

Thu, 16Â AugÂ 2007 00:00:00 -05:00

Background: The restless legs syndrome (RLS) is a common neurologic disorder characterized by an irresistible urge to move the legs. It is a major cause of sleep disruption. Periodic limb...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 24-2007 â A 20-Year-Old Pregnant Woman with Altered Mental Status

Thu, 09Â AugÂ 2007 00:00:00 -05:00

A 20-year-old pregnant woman was admitted to this hospital at 26 weeks of gestation because of dizziness, confusion, and difficulty walking.

Ten weeks before admission, the patient had a positive result...

ORIGINAL ARTICLE: Complement C3 Variant and the Risk of Age-Related Macular Degeneration

Thu, 09Â AugÂ 2007 00:00:00 -05:00

Background: Age-related macular degeneration is the most common cause of blindness in Western populations. Susceptibility is influenced by age and by genetic and environmental factors. Complement activation is implicated in...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Changing the Trajectory of Cognitive Decline?

Marilyn S. Albert, Ph.D. — Thu, 02Â AugÂ 2007 00:00:00 -05:00

Delaying the onset of cognitive decline and reducing cognitive decline once it has begun have become increasingly important with the aging of the population. Age-related changes in cognitive functions such...

IMAGES IN CLINICAL MEDICINE: Macular Hemorrhage from Bungee Jumping

Atul K. Jain, M.D. and Michael Gaynon, M.D. — Thu, 19Â JulÂ 2007 00:00:00 -05:00

A 25-year-old woman with no clinically significant medical history and with normal coagulation and hematologic studies went bungee jumping from a vertical height of 150 ft (45.7 m). Immediately afterward,...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 21-2007 â A 58-Year-Old Woman with Headaches, Weakness, and Strokelike Episodes

Thu, 12Â JulÂ 2007 00:00:00 -05:00

Dr. Kevin N. Sheth (Neurology): A 58-year-old right-handed woman with type 1 diabetes was admitted to the hospital because of a 2-week history of increasing fatigue and word-finding difficulties and...

ORIGINAL ARTICLE: Reduction in Neural-Tube Defects after Folic Acid Fortification in Canada

Thu, 12Â JulÂ 2007 00:00:00 -05:00

Background: In 1998, folic acid fortification of a large variety of cereal products became mandatory in Canada, a country where the prevalence of neural-tube defects was historically higher in the...

CLINICAL PRACTICE: Arteriovenous Malformations of the Brain

Robert M. Friedlander, M.D. — Thu, 28Â JunÂ 2007 00:00:00 -05:00

A 51-year-old woman presents with a generalized tonicâclonic seizure. After a brief postictal period, she recovers fully and does not report headache or other neurologic symptoms. She takes no medications...

ORIGINAL ARTICLE: Allogeneic Bone Marrow Transplantation in Mevalonic Aciduria

Thu, 28Â JunÂ 2007 00:00:00 -05:00

Mevalonic aciduria is a rare, inborn error of isoprene biosynthesis characterized by severe, periodic attacks of fever and inflammation, developmental delay, ataxia, and dysmorphic features. This autosomal recessive disease is...

PERSPECTIVE: Mevalonate Kinase Deficiency and Autoinflammatory Disorders

Thu, 28Â JunÂ 2007 00:00:00 -05:00

A deficiency of mevalonate kinase resulting in mevalonic aciduria was the first inherited defect in cholesterol and nonsterol isoprene biosynthesis to be recognized.1 Nine other enzyme deficiencies have since been...

IMAGES IN CLINICAL MEDICINE: Putaminal Necrosis

Sudipta Chattopadhyay, M.B., B.S., M.D. and Puvana Chandra, M.B., B.S. — Thu, 31Â MayÂ 2007 00:00:00 -05:00

A 51-year-old man presented with acute-onset bilateral blindness that had developed during the previous 4 days. He reported having consumed cheap, unbranded whisky in a bout of binge drinking. This...

ORIGINAL ARTICLE: Surgical versus Nonsurgical Treatment for Lumbar Degenerative Spondylolisthesis

Thu, 31Â MayÂ 2007 00:00:00 -05:00

Background: Management of degenerative spondylolisthesis with spinal stenosis is controversial. Surgery is widely used, but its effectiveness in comparison with that of nonsurgical treatment has not been demonstrated in controlled...

ORIGINAL ARTICLE: Surgery versus Prolonged Conservative Treatment for Sciatica

Thu, 31Â MayÂ 2007 00:00:00 -05:00

Background: Lumbar-disk surgery often is performed in patients who have sciatica that does not resolve within 6 weeks, but the optimal timing of surgery is not known.

Methods: We randomly assigned...

PERSPECTIVE: Back Surgery â Who Needs It?

Thu, 31Â MayÂ 2007 00:00:00 -05:00

Back surgery is not the final common pathway for everyone with persistent back pain. It offers specific therapy for specific anatomical derangements associated with specific complexes of symptoms. When surgery...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 14-2007 â A 59-Year-Old Man with Fever and Pain and Swelling of Both Eyes and the Right Ear

Thu, 10Â MayÂ 2007 00:00:00 -05:00

Dr. David T. Ting (Medicine): A 59-year-old man was admitted to this hospital because of fever as well as pain and swelling of both eyes, which was worse in the...

IMAGES IN CLINICAL MEDICINE: Severe Proliferative Diabetic Retinopathy

Ioannis K. Petropoulos, M.D. and John X. Koliopoulos, M.D., Ph.D. — Thu, 10Â MayÂ 2007 00:00:00 -05:00

A 44-year-old woman with a 20-year history of type 1 diabetes mellitus presented with progressive visual loss over a period of 3 months in both eyes. She had no prior...

ORIGINAL ARTICLE: Long-Term Effect of Diabetes and Its Treatment on Cognitive Function

Thu, 03Â MayÂ 2007 00:00:00 -05:00

Background: Long-standing concern about the effects of type 1 diabetes on cognitive ability has increased with the use of therapies designed to bring glucose levels close to the nondiabetic range...

ORIGINAL ARTICLE: A Lethal Defect of Mitochondrial and Peroxisomal Fission

Thu, 26Â AprÂ 2007 00:00:00 -05:00

We report on a newborn girl with microcephaly, abnormal brain development, optic atrophy and hypoplasia, persistent lactic acidemia, and a mildly elevated plasma concentration of very-long-chain fatty acids. We found...

IMAGES IN CLINICAL MEDICINE: Meningioma

S. Nizam Ahmed, M.D. and James Scozzafava, M.D. — Thu, 19Â AprÂ 2007 00:00:00 -05:00

An 83-year-old healthy woman was admitted with episodes of nausea and vertigo that had increased in frequency over several months. Many of the episodes involved brief periods of unresponsiveness and...

ORIGINAL ARTICLE: Targeted Therapy for Inherited GPI Deficiency

Thu, 19Â AprÂ 2007 00:00:00 -05:00

Disrupted binding of the transcription factor Sp1 to the mutated promoter region of the mannosyl transferaseâencoding gene PIGM causes inherited glycosylphosphatidylinositol (GPI) deficiency characterized by splanchnic vein thrombosis and epilepsy....

PERSPECTIVE: Approving the Vagus-Nerve Stimulator for Depression

Thu, 19Â AprÂ 2007 00:00:00 -05:00

The vagus-nerve stimulator (VNS), a device that is implanted by a neurosurgeon and sends intermittent electrical pulses to the brain, has been marketed in the United States since 1997 as...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Retinal Progenitor Cells â Timing Is Everything

Jean Bennett, M.D., Ph.D. — Thu, 12Â AprÂ 2007 00:00:00 -05:00

More than 10 million people in the United States are blind or visually impaired, and 50,000 more will become blind each year. The results of a survey conducted by the...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 11-2007 â A 59-Year-Old Man with Neck Pain, Weakness in the Arms, and Cranial-Nerve Palsies

Thu, 12Â AprÂ 2007 00:00:00 -05:00

A 59-year-old man was admitted to this hospital in late summer because of back pain, weakness in the right arm, and cranial-nerve palsies.

The patient was in his usual state of...

ORIGINAL ARTICLE: Radiotherapy for Glioblastoma in the Elderly

Thu, 12Â AprÂ 2007 00:00:00 -05:00

Background: There is no community standard for the treatment of glioblastoma in patients 70 years of age or older. We conducted a randomized trial that compared radiotherapy and supportive care...

ORIGINAL ARTICLE: Mutations in Associated with Neural-Tube Defects

Thu, 05Â AprÂ 2007 00:00:00 -05:00

Neural-tube defects such as anencephaly and spina bifida constitute a group of common congenital malformations caused by complex genetic and environmental factors. We have identified three mutations in the VANGL1...

IMAGES IN CLINICAL MEDICINE: Dupuytren's Contracture

Edward Calif, M.D. and Shalom Stahl, M.D. — Thu, 22Â MarÂ 2007 00:00:00 -05:00

A 57-year-old man with a history of diabetes mellitus and alcohol consumption was referred to the hand surgery unit owing to contractures of fingers of both hands. He reported that...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 9-2007 â A 27-Year-Old Woman with Pain and Swelling of the Legs

Jean-Michel Vallat, M.D., Didier P. Cros, M.D., and E. Tessa Hedley-Whyte, M.D. — Thu, 22Â MarÂ 2007 00:00:00 -05:00

A 27-year-old woman was seen in the neurology clinic because of pain and swelling in her feet. She had been well until approximately 5 months earlier, when swelling of her...

ORIGINAL ARTICLE: Neurodevelopment and Cognition in Children after Enterovirus 71 Infection

Thu, 22Â MarÂ 2007 00:00:00 -05:00

Background: Enterovirus 71 is a common cause of hand, foot, and mouth disease and encephalitis in Asia and elsewhere. The long-term neurologic and psychiatric effects of this viral infection on...

PERSPECTIVE: Enterovirus DÃ©jÃ Vu

Thu, 22Â MarÂ 2007 00:00:00 -05:00

More than 90 human enterovirus serotypes have now been identified in three distinct waves of discovery. The three poliovirus serotypes were first isolated from nonhuman primates in the course of...

IMAGES IN CLINICAL MEDICINE: Calcified Left Atrial Thrombus

Joseph M. Pappachan, M.D. and Benjamin C. Bino, M.D. — Thu, 08Â MarÂ 2007 00:00:00 -05:00

A 21-year-old woman with a history of rheumatic fever at 7 years of age presented with left-sided weakness. A large ischemic infarction involving the territory of the right middle cerebral...

CLINICAL PRACTICE: Pediatric Strabismus

Sean P. Donahue, M.D., Ph.D. — Thu, 08Â MarÂ 2007 00:00:00 -05:00

A healthy 3-year-old boy presents with a 6-month history of strabismus in his left eye. The visible inward deviation of the eye began intermittently but is now constant. His visual...

IMAGES IN CLINICAL MEDICINE: Central Retinal Artery Occlusion

Kayvan Arashvand, M.D. — Thu, 22Â FebÂ 2007 00:00:00 -05:00

A 76-year-old man with hypertension presented with a sudden, painless, and profound loss of vision in the right eye. Visual acuity was light perception only in the affected eye, with...

ORIGINAL ARTICLE: Missense Mutations in the Gene as a Cause of the BjÃ¶rnstad Syndrome

Thu, 22Â FebÂ 2007 00:00:00 -05:00

Background: The BjÃ¶rnstad syndrome, an autosomal recessive disorder associated with sensorineural hearing loss and pili torti, is caused by mutation of a previously unidentified gene on chromosome 2q34â36.

Methods: Refined genetic...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 4-2007 â A 56-Year-Old Woman with Rapidly Progressive Vertigo and Ataxia

Thu, 08Â FebÂ 2007 00:00:00 -05:00

A 56-year-old woman was admitted to the hospital because of rapidly progressive vertigo and ataxia.

The patient had been well until approximately 10 weeks before admission, when occasional dizziness and nausea...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Huntington's Disease â Making Connections

J. Timothy Greenamyre, M.D., Ph.D. — Thu, 01Â FebÂ 2007 00:00:00 -05:00

When the causative gene for Huntington's disease was identified in 1993, there was great anticipation and hope that key disease-causing mechanisms would be identified quickly and that rational neuroprotective treatments...

IMAGES IN CLINICAL MEDICINE: A Medical Mystery: Dilated Bowel

Jason Handwerker, M.D. and Vassilios D. Raptopoulos, M.D. — Thu, 01Â FebÂ 2007 00:00:00 -05:00

A 70-year-old man presented with a history of increasing abdominal distention. On physical examination, the patient was afebrile and his abdomen was soft and nontender. An abdominal radiograph raised concern...

EDITORIAL: The End of an Era in Otitis Research

Stephen Berman, M.D. — Thu, 18Â JanÂ 2007 00:00:00 -05:00

Retrospective studies carried out in the 1950s, 1960s, and 1970s suggested an association between otitis media early in life and subsequent developmental impairments in children. Although these studies were not...

ORIGINAL ARTICLE: Tympanostomy Tubes and Developmental Outcomes at 9 to 11 Years of Age

Thu, 18Â JanÂ 2007 00:00:00 -05:00

Background: Developmental impairments in children have been attributed to persistent middle-ear effusion in their early years of life. Previously, we reported that among children younger than 3 years of age...

IMAGES IN CLINICAL MEDICINE: Complication of Central Venous Catheterization

Hangyuan Guo, M.D. — Thu, 11Â JanÂ 2007 00:00:00 -05:00

A 40-year-old man with Crohn's disease underwent an uncomplicated operation involving lysis of adhesions that were causing intestinal obstruction. After surgery, a cardiologist inserted a central venous catheter through the...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 1-2007 â A 40-Year-Old Woman with Epistaxis, Hematemesis, and Altered Mental Status

Thu, 11Â JanÂ 2007 00:00:00 -05:00

Dr. Amy Jo Chien (Medicine): A 40-year-old woman was admitted to the emergency department of this hospital because of the sudden onset of headache, loss of consciousness, and coagulopathy.

The patient...

CLINICAL PRACTICE: Concussion

Allan H. Ropper, M.D., and Kenneth C. Gorson, M.D. — Thu, 11Â JanÂ 2007 00:00:00 -05:00

A 64-year-old woman slipped on an icy walk, falling forward and striking her forehead. She had a brief convulsion immediately after the fall, was unresponsive for less than 1 minute,...

CLINICAL PROBLEM-SOLVING: A Stain in Time

Thu, 04Â JanÂ 2007 00:00:00 -05:00

A 45-year-old woman from northern Ontario presented to her local hospital with a 2-year history of asymmetric migratory arthralgias involving the left knee, ankles, elbows, and fingers. She also had...

PERSPECTIVE: Drugs and Valvular Heart Disease

Thu, 04Â JanÂ 2007 00:00:00 -05:00

In 1997, Connolly et al. reported that both racemic fenfluramine (Pondimin) and dexfenfluramine (Redux) were associated with valvular heart disease.1 The valvular abnormalities seen in patients treated with these agents...

REVIEW ARTICLE: Whipple's Disease

Thu, 04Â JanÂ 2007 00:00:00 -05:00

The discovery of Whipple's disease and its causative bacterium, Tropheryma whipplei, is a prime example of how modern technologies have contributed to medical knowledge. Although Whipple's disease was first described...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: The Persistence of Memory

Mark R. Cookson, Ph.D., and John Hardy, Ph.D. — Thu, 21Â DecÂ 2006 00:00:00 -05:00

We often do not realize how much we need something until it is gone â and Alzheimer's disease shows us how debilitating it is to lose memory. Although we now...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 39-2006 â A 24-Year-Old Woman with Systemic Lupus Erythematosus, Seizures, and Right Arm Weakness

Thu, 21Â DecÂ 2006 00:00:00 -05:00

Dr. Sherry Chou (Neurology): A 24-year-old woman was transferred to this hospital because of seizures and a lesion in the brain.

A diagnosis of systemic lupus erythematosus (SLE) had been made...

ORIGINAL ARTICLE: PET of Brain Amyloid and Tau in Mild Cognitive Impairment

Thu, 21Â DecÂ 2006 00:00:00 -05:00

Background: Amyloid senile plaques and tau neurofibrillary tangles are neuropathological hallmarks of Alzheimer's disease that accumulate in the cortical regions of the brain in persons with mild cognitive impairment who...

PERSPECTIVE: Reconstructing Lives â A Tale of Two Soldiers

Thu, 21Â DecÂ 2006 00:00:00 -05:00

Jason Pepper can't see the deer and wild turkeys that feed in the pasture in front of his new home, an hour's drive from Nashville. But when he sits and...

IMAGES IN CLINICAL MEDICINE: Abnormal Eye Movements Associated with Unilateral Loss of Vestibular Function

Richard F. Lewis, M.D. and John P. Carey, M.D. — Thu, 14Â DecÂ 2006 00:00:00 -05:00

Acute unilateral loss of peripheral vestibular function causes spontaneous nystagmus that is horizontal and torsional, with slow phases directed toward the abnormal ear. The nystagmus results from an imbalance in...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 38-2006 â A 5-Year-Old Boy with Headache and Abdominal Pain

Thu, 14Â DecÂ 2006 00:00:00 -05:00

Dr. Kerstin Zanger (Pediatrics): A 5-year-old boy was admitted to the hospital because of headache, abdominal pain that had increased in severity and frequency, increasing abdominal girth, and a rash.

The...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Stress, Aging, and Neurodegenerative Disease

Richard I. Morimoto, Ph.D. — Thu, 23Â NovÂ 2006 00:00:00 -05:00

Aging and stress, stress and aging â these two human conditions, when paired, can profoundly affect the quality of life. When events go awry, molecular processes take place that, over...

REVIEW ARTICLE: Spinal Epidural Abscess

Rabih O. Darouiche, M.D. — Thu, 09Â NovÂ 2006 00:00:00 -05:00

Despite advances in medical knowledge, imaging techniques, and surgical interventions, spinal epidural abscess remains a challenging problem that often eludes diagnosis and receives suboptimal treatment. The incidence of this disease...

ORIGINAL ARTICLE: Pallidal Deep-Brain Stimulation in Primary Generalized or Segmental Dystonia

Thu, 09Â NovÂ 2006 00:00:00 -05:00

Background: Neurostimulation of the internal globus pallidus has been shown to be effective in reducing symptoms of primary dystonia. We compared this surgical treatment with sham stimulation in a randomized,...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: A Reversal of Misfortune for Myotonic Dystrophy?

Thomas A. Cooper, M.D. — Thu, 26Â OctÂ 2006 00:00:00 -05:00

Myotonic dystrophy, the most common cause of adult-onset muscular dystrophy, is a dominantly inherited disorder in which death is usually caused by the wasting of skeletal muscle and defects in...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Amyotrophic Lateral Sclerosis â Are Microglia Killing Motor Neurons?

David R. Borchelt, Ph.D. — Thu, 12Â OctÂ 2006 00:00:00 -05:00

Amyotrophic lateral sclerosis (ALS) is a disease characterized by the selective degeneration of motor neurons. The only known genetic cause of the disease, which accounts for about 2% of cases,...

IMAGES IN CLINICAL MEDICINE: Medical Mystery â Visual-Field Defect

Jeevan R. Mathura, Jr., M.D. and Lee M. Jampol, M.D. — Thu, 12Â OctÂ 2006 00:00:00 -05:00

A 31-year-old woman presented with a sudden onset of an inferonasal visual-field deficit in the left eye 2 days after she had traveled 16 hours by air. Physical examination on...

EDITORIAL: A Very Effective Treatment for Neovascular Macular Degeneration

Edwin M. Stone, M.D., Ph.D. — Thu, 05Â OctÂ 2006 00:00:00 -05:00

Age-related macular degeneration, a potentially blinding disease, is now epidemic in the developed world. Roughly one in three people will be affected to some degree by the age of 75...

CLINICAL PROBLEM-SOLVING: Lost in Transcription

Thu, 05Â OctÂ 2006 00:00:00 -05:00

Urinary urgency and fever developed in a 55-year-old, bedridden woman with multiple sclerosis, a long-term indwelling Foley catheter, and multiple prior urinary tract infections. The patient had recently been transferred...

REVIEW ARTICLE: Age-Related Macular Degeneration

Paulus T.V.M. de Jong, M.D., Ph.D. — Thu, 05Â OctÂ 2006 00:00:00 -05:00

Since 1874, when it was first described in the medical literature as âsymmetrical central choroido-retinal disease occurring in senile persons,â1 age-related macular degeneration has also been referred to as senile,...

ORIGINAL ARTICLE: Ranibizumab versus Verteporfin for Neovascular Age-Related Macular Degeneration

Thu, 05Â OctÂ 2006 00:00:00 -05:00

Background: We compared ranibizumab â a recombinant, humanized, monoclonal antibody Fab that neutralizes all active forms of vascular endothelial growth factor A â with photodynamic therapy with verteporfin in the...

ORIGINAL ARTICLE: Ranibizumab for Neovascular Age-Related Macular Degeneration

Thu, 05Â OctÂ 2006 00:00:00 -05:00

Background: Ranibizumab â a recombinant, humanized, monoclonal antibody Fab that neutralizes all active forms of vascular endothelial growth factor A â has been evaluated for the treatment of neovascular age-related...

PERSPECTIVE: The Price of Sight â Ranibizumab, Bevacizumab, and the Treatment of Macular Degeneration

Thu, 05Â OctÂ 2006 00:00:00 -05:00

On June 30, 2006, the Food and Drug Administration (FDA) approved ranibizumab â which is manufactured by Genentech and marketed as Lucentis â for the treatment of neovascular age-related macular...

VIDEOS IN CLINICAL MEDICINE: Lumbar Puncture

Thu, 28Â SepÂ 2006 00:00:00 -05:00

...

REVIEW ARTICLE: The Tuberous Sclerosis Complex

Thu, 28Â SepÂ 2006 00:00:00 -05:00

The tuberous sclerosis complex (TSC), a multisystem, autosomal dominant disorder affecting children and adults, results from mutations in one of two genes, TSC1 (encoding hamartin) or TSC2 (encoding tuberin) (see...

IMAGES IN CLINICAL MEDICINE: Regression of Optociliary Shunt Vessels

Adrienne Ruth, M.D. and Nancy J. Newman, M.D. — Thu, 21Â SepÂ 2006 00:00:00 -05:00

A previously healthy 53-year-old man presented with headache, blurred vision, and pulsatile tinnitus. A lumbar puncture demonstrated meningitis with increased intracranial pressure (opening pressure, 37 cm H2O), with a white-cell...

REVIEW ARTICLE: Cerebral Aneurysms

Jonathan L. Brisman, M.D., Joon K. Song, M.D., and David W. Newell, M.D. — Thu, 31Â AugÂ 2006 00:00:00 -05:00

Saccular intracranial aneurysms, abnormal focal outpouchings of cerebral arteries, cause substantial rates of morbidity and mortality. Recently, major changes have occurred in the way we think about and treat this...

ORIGINAL ARTICLE: A Randomized Trial of Deep-Brain Stimulation for Parkinson's Disease

Thu, 31Â AugÂ 2006 00:00:00 -05:00

Background: Neurostimulation of the subthalamic nucleus reduces levodopa-related motor complications in advanced Parkinson's disease. We compared this treatment plus medication with medical management.

Methods: In this randomized-pairs trial, we enrolled 156...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 26-2006 â A 19-Year-Old Woman with Difficulty Walking

Nagagopal Venna, M.D., Katherine B. Sims, M.D., and P. Ellen Grant, M.D. — Thu, 24Â AugÂ 2006 00:00:00 -05:00

Dr. Roy N. Alcalay (Neurology): A 19-year-old right-handed woman was referred to the neurology clinic because of slowly worsening difficulty walking and painful spasms in her legs and arms. When...

REVIEW ARTICLE: Dystonia

Daniel Tarsy, M.D., and David K. Simon, M.D., Ph.D. — Thu, 24Â AugÂ 2006 00:00:00 -05:00

The term âdystoniaâ was coined by Oppenheim in 1911 to describe a disorder causing variable muscle tone and recurrent muscle spasm. This disorder was initially called dystonia musculorum deformans12 and...

EDITORIAL: Neuroimaging and the Prediction of Outcomes in Preterm Infants

Olaf Dammann, M.D., and Alan Leviton, M.D. — Thu, 17Â AugÂ 2006 00:00:00 -05:00

Among the most pressing questions for the parents and caregivers of extremely preterm infants are those about their children's future. Will they survive? If so, what are their chances of...

ORIGINAL ARTICLE: Neonatal MRI to Predict Neurodevelopmental Outcomes in Preterm Infants

Thu, 17Â AugÂ 2006 00:00:00 -05:00

Background: Very preterm infants are at high risk for adverse neurodevelopmental outcomes. Magnetic resonance imaging (MRI) has been proposed as a means of predicting neurodevelopmental outcomes in this population.

Methods: We...

IMAGES IN CLINICAL MEDICINE: Adie's Pupil in the Ross Syndrome

Georg Hagemann, M.D. and Thomas Bartke, M.D. — Thu, 10Â AugÂ 2006 00:00:00 -05:00

A 34-year-old woman reported that the right side of her face was warmer than the left and had an unusual propensity for sweating. (Panels A and B were obtained after...

ORIGINAL ARTICLE: Neonatal-Onset Multisystem Inflammatory Disease Responsive to Interleukin-1Î² Inhibition

Thu, 10Â AugÂ 2006 00:00:00 -05:00

Background: Neonatal-onset multisystem inflammatory disease is characterized by fever, urticarial rash, aseptic meningitis, deforming arthropathy, hearing loss, and mental retardation. Many patients have mutations in the cold-induced autoinflammatory syndrome 1...

IMAGES IN CLINICAL MEDICINE: Lung Carcinoma with Anti-Hu Paraneoplastic Syndrome

William C. Gordon, M.R.C.P. and Michael T.J. Leach, M.R.C.Path. — Thu, 03Â AugÂ 2006 00:00:00 -05:00

A 54-year-old female smoker presented with a three-month history of anorexia, fatigue, weight loss, dizziness, and a burning sensation in her hands and feet. She had marked postural hypotension. Her...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 23-2006 â A 36-Year-Old Man with Numbness in the Right Hand and Hypertension

Thu, 27Â JulÂ 2006 00:00:00 -05:00

Dr. Joanna J. Wykrzykowska (Medicine): A 36-year-old man was admitted to the hospital because of a mass in the right adrenal gland. The patient had been well until seven months...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 22-2006 â A 77-Year-Old Man with a Rapidly Progressive Gait Disorder

Thu, 20Â JulÂ 2006 00:00:00 -05:00

Dr. Robert H. Ackerman: A 77-year-old man was admitted to this hospital because of recent episodes of falling. He had been well until 18 months earlier, when an abscess of...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 21-2006 â A 61-Year-Old Man with Left-Sided Facial Pain

Emad Eskandar, M.D., Fred G. Barker II, M.D., and James D. Rabinov, M.D. — Thu, 13Â JulÂ 2006 00:00:00 -05:00

A 61-year-old man was seen in a specialty clinic at this hospital because of facial pain. The patient had been in his usual state of health until eight months earlier,...

IMAGES IN CLINICAL MEDICINE: Abdominal Pseudohernia Due to Herpes Zoster

N. Troy Tagg, M.D. and Jack W. Tsao, M.D., D.Phil. — Thu, 06Â JulÂ 2006 00:00:00 -05:00

A vesicular rash of the right T11âT12 dermatomes developed in a 75-year-old man. Four weeks later, as the rash was resolving (Panel A), he noticed a protrusion of the right...

CLINICAL PROBLEM-SOLVING: Heading Down the Wrong Path

Thu, 06Â JulÂ 2006 00:00:00 -05:00

A 52-year-old woman who had undergone renal transplantation 13 years earlier was brought by her family to the emergency department of a community hospital. Five days earlier, she had suddenly...

IMAGES IN CLINICAL MEDICINE: Frey's Syndrome

Francesco Prattico, M.D. and Paola Perfetti, M.D. — Thu, 06Â JulÂ 2006 00:00:00 -05:00

A 30-year-old man had a pleomorphic adenoma removed from his left parotid gland. His postoperative course was uncomplicated. Two months later, he noted that his left cheek became wet while...

IMAGES IN CLINICAL MEDICINE: Hypothyroid-Induced Hyporeflexia

Patrick R. Sosnay, M.D. and Scott Kim, M.D. — Thu, 29Â JunÂ 2006 00:00:00 -05:00

A 48-year-old woman was brought to the hospital by her family because of diffuse, nonspecific pain. She was unable to provide her medical history. During the first hospital day, hypothermia...

EDITORIAL: Vitamin B, Folic Acid, and the Prevention of Dementia

Robert Clarke, M.D. — Thu, 29Â JunÂ 2006 00:00:00 -05:00

Finding treatments for the prevention of dementia is an important challenge for medical research.1 Dementia currently affects about 4.5 million persons in the United States, and many more have cognitive...

IMAGES IN CLINICAL MEDICINE: Disseminated Central Nervous System Nocardiosis

Megdad Zaatreh, M.D. and Wael Alabulkarim, M.D. — Thu, 29Â JunÂ 2006 00:00:00 -05:00

A 55-year-old man presented with a two-day history of headache, fever, and generalized weakness. He had received a cadaveric kidney transplant five years earlier. His medications included 5 mg of...

ORIGINAL ARTICLE: A Controlled Trial of Homocysteine Lowering and Cognitive Performance

Thu, 29Â JunÂ 2006 00:00:00 -05:00

Background: The results of observational studies suggest that plasma homocysteine concentrations are inversely related to cognitive function in older people. Our objective was to test the hypothesis that lowering the...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 18-2006 â A 57-Year-Old Woman with Numbness and Weakness of the Feet and Legs

Thu, 15Â JunÂ 2006 00:00:00 -05:00

A 57-year-old woman was seen in the neurology clinic of this hospital because of longtime numbness and weakness in her feet and legs.

In adolescence, she had noticed that her pupils...

CLINICAL PROBLEM-SOLVING: Thinking outside the Box

Ramin Farzaneh-Far, M.D., Talya Schwarzberg, M.D., and Stuart B. Mushlin, M.D. — Thu, 01Â JunÂ 2006 00:00:00 -05:00

A 35-year-old man with advanced acquired immunodefiency syndrome (AIDS) presented to the emergency department after a witnessed syncopal event. He had noted light-headedness after walking from the bathroom to his...

IMAGES IN CLINICAL MEDICINE: Sympathetic-Nerve Damage in Brachial-Plexus Palsy

Israel Alfonso, M.D. and Ilker Yaylali, M.D., Ph.D. — Thu, 18Â MayÂ 2006 00:00:00 -05:00

A mother brought her three-month-old infant to the clinic because of a persistent difference in temperature between the right and left hand. The child had had a total brachial-plexus palsy...

ORIGINAL ARTICLE: Language Ability after Early Detection of Permanent Childhood Hearing Impairment

Thu, 18Â MayÂ 2006 00:00:00 -05:00

Background: Children with bilateral permanent hearing impairment often have impaired language and speech abilities. However, the effects of universal newborn screening for permanent bilateral childhood hearing impairment and the effects...

EDITORIAL: Neonatal Hyperbilirubinemia â What Are the Risks?

Jon F. Watchko, M.D. — Thu, 04Â MayÂ 2006 00:00:00 -05:00

Hyperbilirubinemia is the most common condition requiring evaluation and treatment of newborns, but for the vast majority of neonates, it is a benign, transitional phenomenon of no overt clinical significance....

ORIGINAL ARTICLE: Outcomes among Newborns with Total Serum Bilirubin Levels of 25 mg per Deciliter or More

Thu, 04Â MayÂ 2006 00:00:00 -05:00

Background: The neurodevelopmental risks associated with high total serum bilirubin levels in newborns are not well defined.

Methods: We identified 140 infants with neonatal total serum bilirubin levels of at least...

ORIGINAL ARTICLE: Recessive Symptomatic Focal Epilepsy and Mutant Contactin-Associated Protein-like 2

Thu, 30Â MarÂ 2006 00:00:00 -05:00

Contactin-associated protein-like 2 (CASPR2) is encoded by CNTNAP2 and clusters voltage-gated potassium channels (Kv1.1) at the nodes of Ranvier. We report a homozygous mutation of CNTNAP2 in Old Order Amish...

CLINICAL PRACTICE: Optic Neuritis

Laura J. Balcer, M.D., M.S.C.E. — Thu, 23Â MarÂ 2006 00:00:00 -05:00

A 25-year-old woman presents with a one-week history of blurred vision in the left eye. One day before the onset of visual loss, she noted a dull ache in the...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 5-2006 â An 11-Year-Old Girl with Loss of Vision in the Right Eye

Thu, 16Â FebÂ 2006 00:00:00 -05:00

Dr. Torsten Wiegand (Massachusetts Eye and Ear Infirmary): An 11-year-old girl was evaluated at our institution because of a loss of vision in the right eye.

One month earlier, she had...

PERSPECTIVE: Stroke and Neurovascular Protection

Thu, 09Â FebÂ 2006 00:00:00 -05:00

Neurons are extremely sensitive cells, whose function, like that of all cells, can be influenced by changes in their environment. Using pumps to regulate the internal and external electrolyte milieu,...

REVIEW ARTICLE: Aneurysmal Subarachnoid Hemorrhage

Jose I. Suarez, M.D., Robert W. Tarr, M.D., and Warren R. Selman, M.D. — Thu, 26Â JanÂ 2006 00:00:00 -05:00

Nontraumatic subarachnoid hemorrhage is a neurologic emergency characterized by the extravasation of blood into the spaces covering the central nervous system that are filled with cerebrospinal fluid. The leading cause...

CLINICAL PRACTICE: Chronic Daily Headache

David W. Dodick, M.D. — Thu, 12Â JanÂ 2006 00:00:00 -05:00

A 36-year-old woman with a long history of catamenial migraines had had a headache almost every day during the previous year. The background headache was mild but became severe and...

IMAGES IN CLINICAL MEDICINE: PellegriniâStieda Syndrome

Eric L. Altschuler, M.D., Ph.D. and Thomas N. Bryce, M.D. — Thu, 05Â JanÂ 2006 00:00:00 -05:00

A 38-year-old man with an incomplete spinal cord injury secondary to a diving accident some years before, which caused tetraplegia but preserved some sensation below the neck, reported new pain...

REVIEW ARTICLE: Community-Acquired Bacterial Meningitis in Adults

Thu, 05Â JanÂ 2006 00:00:00 -05:00

Bacterial meningitis is a medical, neurologic, and sometimes neurosurgical emergency that requires a multidisciplinary approach. Bacterial meningitis has an annual incidence of 4 to 6 cases per 100,000 adults (defined...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 36-2005 â A 61-Year-Old Woman with Seizure, Disturbed Gait, and Altered Mental Status

Thu, 24Â NovÂ 2005 00:00:00 -05:00

Dr. Ronan Walsh (Department of Neurology, Brigham and Women's Hospital): A 61-year-old left-handed woman was admitted to the neurology service of this hospital because of a seizure and altered mental...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 34-2005 â A 10-Year-Old Girl with a Bullous Skin Eruption and Acute Respiratory Failure

Robert L. Sheridan, M.D., Vincent Liu, M.D., and Sudha Anupindi, M.D. — Thu, 10Â NovÂ 2005 00:00:00 -05:00

A 10-year-old girl was transferred to the Shriners Hospital for Children because of a bullous skin eruption, with sloughing of the skin and respiratory failure.

The patient had been well until...

IMAGES IN CLINICAL MEDICINE: Traumatic Cranial Nerve Palsy

Christopher Baker, M.D. and Jeremy Cannon, M.D. — Thu, 03Â NovÂ 2005 00:00:00 -05:00

A 19-year-old man sustained a crush injury to his skull from a piece of heavy machinery. Although on initial assessment he readily followed commands, his mental status deteriorated and he...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Tangles and Neurodegenerative Disease â A Surprising Twist

Rudolph E. Tanzi, Ph.D. — Thu, 27Â OctÂ 2005 00:00:00 -05:00

Nearly 100 years ago, a group of Bavarian psychiatrists gathered in a small conference hall in TÃ¼bingen to hear a presentation by Dr. Alois Alzheimer. To their surprise, he proposed...

IMAGES IN CLINICAL MEDICINE: Left Sixth Cranial Nerve Palsy with Herpes Zoster Ophthalmicus

Edward Jude, M.D., M.R.C.P. and Amiya Chakraborty, M.R.C.P. — Thu, 20Â OctÂ 2005 00:00:00 -05:00

A 62-year-old man reported acute left retro-orbital pain of one week's duration. Physical examination revealed no abnormalities. Three days later, double vision developed, and the next day a rash appeared...

ORIGINAL ARTICLE: Severe Expressive-Language Delay Related to Duplication of the WilliamsâBeuren Locus

Thu, 20Â OctÂ 2005 00:00:00 -05:00

The WilliamsâBeuren syndrome (WBS) locus, at 7q11.23, is prone to recurrent chromosomal rearrangements, including the microdeletion that causes WBS, a multisystem condition with characteristic cardiovascular, cognitive, and behavioral features. It...

PERSPECTIVE: On Genes, Speech, and Language

Simon E. Fisher, D.Phil. — Thu, 20Â OctÂ 2005 00:00:00 -05:00

Learning to talk is one of the most important milestones in human development, but we still have only a limited understanding of the way in which the process occurs. It...

EDITORIAL: Systemic Hypothermia â A âCoolâ Therapy for Neonatal HypoxicâIschemic Encephalopathy

Lu-Ann Papile, M.D. — Thu, 13Â OctÂ 2005 00:00:00 -05:00

Hypoxicâischemic encephalopathy is an important cause of death and disability in full-term infants. The incidence of moderate or severe hypoxicâischemic encephalopathy has remained essentially unchanged over the past 20 years,...

ORIGINAL ARTICLE: Plasma Exchange for Primary Autoimmune Autonomic Failure

Thu, 13Â OctÂ 2005 00:00:00 -05:00

We report on a patient with long-standing severe autonomic failure that affected his sympathetic and parasympathetic nervous systems. Antibodies against the ganglionic acetylcholine receptors were detected in the serum. Removal...

ORIGINAL ARTICLE: Whole-Body Hypothermia for Neonates with HypoxicâIschemic Encephalopathy

Thu, 13Â OctÂ 2005 00:00:00 -05:00

Background: Hypothermia is protective against brain injury after asphyxiation in animal models. However, the safety and effectiveness of hypothermia in term infants with encephalopathy is uncertain.

Methods: We conducted a randomized...

IMAGES IN CLINICAL MEDICINE: Medical Mystery â One Brown Eye and One Blue Eye

Benjamin Gesundheit, M.D., Ph.D. and Mark Greenberg, M.D. — Thu, 06Â OctÂ 2005 00:00:00 -05:00

A 10-year-old boy has a brown right eye and a blue left eye, as well as mild ptosis and miosis of his left eye. What is the condition he had...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Anchors Away â Of Plaques and Pathology in Prion Disease

Glenn Telling, Ph.D. — Thu, 15Â SepÂ 2005 00:00:00 -05:00

The accumulation of conformationally altered cellular proteins is a common feature underlying the pathogenesis of neurodegenerative conditions such as Alzheimer's disease and the prion diseases. However, the proteins that cause...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 27-2005 â An 80-Year-Old Man with Fatigue, Unsteady Gait, and Confusion

Thu, 08Â SepÂ 2005 00:00:00 -05:00

Dr. Thanh Nguyen (Neurology): An 80-year-old man was admitted to the hospital in late summer because of difficulty walking, fatigue, confusion, and insomnia.

The patient had been in his usual state...

IMAGES IN CLINICAL MEDICINE: Roth's Spots

Zsolt Varga, M.B., B.Chir. and Jiri Pavlu, M.D. — Thu, 08Â SepÂ 2005 00:00:00 -05:00

A 24-year-old man presented with a three-day history of a blurred patch near the center of his left visual field. He reported no fatigue or weight loss. Ophthalmoscopy showed venous...

IMAGES IN CLINICAL MEDICINE: Air-Pressure Injury of the Eye and Intracranial Structures

Stefan Mennel, M.D. and Silvia Peter, M.D. — Thu, 01Â SepÂ 2005 00:00:00 -05:00

A 5-year-old boy activated an air-pressure pistol from a distance of about 10 cm, pointing directly toward his right eye. Examination revealed downward vertical deviation of the right eye and...

REVIEW ARTICLE: Neural Stem Cells and the Origin of Gliomas

Nader Sanai, M.D., Arturo Alvarez-Buylla, Ph.D., and Mitchel S. Berger, M.D. — Thu, 25Â AugÂ 2005 00:00:00 -05:00

Despite progress in research on the molecular aspects of malignant gliomas, the prognosis of these brain tumors continues to be dismal. In glioblastoma, the most common glioma variant in adults,...

CLINICAL PRACTICE: Chronic Insomnia

Michael H. Silber, M.B., Ch.B. — Thu, 25Â AugÂ 2005 00:00:00 -05:00

A 46-year-old woman has difficulty in falling asleep and staying asleep. The problem started after the birth of her second child 15 years earlier in association with mild postpartum depression....

IMAGES IN CLINICAL MEDICINE: Central Serous Chorioretinopathy in Pregnancy

George L. Mayo, M.D. and Michael J. Tolentino, M.D. — Thu, 18Â AugÂ 2005 00:00:00 -05:00

A 32-year-old black woman who was seven months pregnant presented with a three-day history of blurring and greenish discoloration of her central vision in the left eye. Visual acuity was...

EDITORIAL: Shift-Work Sleep Disorder â The Glass Is More Than Half Empty

Robert C. Basner, M.D. — Thu, 04Â AugÂ 2005 00:00:00 -05:00

As many as 20 percent of workers in industrialized nations are shift workers â in other words, people who work either at night or on rotating shifts.1 Shift-work sleep disorder,...

ORIGINAL ARTICLE: Modafinil for Excessive Sleepiness Associated with Shift-Work Sleep Disorder

Thu, 04Â AugÂ 2005 00:00:00 -05:00

Background: Patients with shift-work sleep disorder chronically have excessive sleepiness during night work and insomnia when attempting to sleep during the day. We evaluated the use of modafinil for treating...

ORIGINAL ARTICLE: Progressive Multifocal Leukoencephalopathy in a Patient Treated with Natalizumab

Thu, 28Â JulÂ 2005 00:00:00 -05:00

We describe the clinical course of a patient with multiple sclerosis in whom progressive multifocal leukoencephalopathy (PML), an opportunistic viral infection of the central nervous system, developed during treatment with...

ORIGINAL ARTICLE: Progressive Multifocal Leukoencephalopathy Complicating Treatment with Natalizumab and Interferon Beta-1a for Multiple Sclerosis

B.K. Kleinschmidt-DeMasters, M.D., and Kenneth L. Tyler, M.D. — Thu, 28Â JulÂ 2005 00:00:00 -05:00

A 46-year-old woman with relapsingâremitting multiple sclerosis died from progressive multifocal leukoencephalopathy (PML) after having received 37 doses of natalizumab (300 mg every four weeks) as part of a clinical...

IMAGES IN CLINICAL MEDICINE: Gnathostomiasis â Neuroimaging of Larval Migration

Pasiri Sithinamsuwan, M.D. and Parnsiri Chairangsaris, M.D. — Thu, 14Â JulÂ 2005 00:00:00 -05:00

An 18-year-old man presented with symptoms that evolved over the course of three days and included fever, radicular back pain, vertigo, ataxia, headache, and left-sided hemiparesis. He reported that he...

EDITORIAL: Inhaled Nitric Oxide for Preterm Infants â Who Benefits?

Richard J. Martin, M.B., F.R.A.C.P., and Michele C. Walsh, M.D. — Thu, 07Â JulÂ 2005 00:00:00 -05:00

Endogenously released nitric oxide is widely recognized to play a key role in multiple vertebrate organ systems, and its deficiency disrupts pulmonary parenchymal and vascular development. These observations have suggested...

ORIGINAL ARTICLE: Neurodevelopmental Outcomes of Premature Infants Treated with Inhaled Nitric Oxide

Thu, 07Â JulÂ 2005 00:00:00 -05:00

Background: Chronic lung disease and severe intraventricular hemorrhage or periventricular leukomalacia in premature infants are associated with abnormal neurodevelopmental outcomes. In a previous randomized, controlled, single-center trial of premature infants...

REVIEW ARTICLE: Î³-Hydroxybutyric Acid

O. Carter Snead III, M.D., and K. Michael Gibson, Ph.D. — Thu, 30Â JunÂ 2005 00:00:00 -05:00

The short-chain fatty acid Î³-hydroxybutyric acid (GHB) was synthesized in 1960 in an attempt to create an analogue of the ubiquitous inhibitory brain neurotransmitter Î³-aminobutyric acid (GABA) that would cross...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 19-2005 â A 17-Year-Old Girl with Respiratory Distress and Hemiparesis after Surviving a Tsunami

Thu, 23Â JunÂ 2005 00:00:00 -05:00

Editor's note: After the earthquake and tsunami in the Indian Ocean on December 26, 2004, the Massachusetts General Hospital formed a clinical-response team to work with Project HOPE (Health Opportunities...

ORIGINAL ARTICLE: Survival after Treatment of Rabies with Induction of Coma

Thu, 16Â JunÂ 2005 00:00:00 -05:00

We report the survival of a 15-year-old girl in whom clinical rabies developed one month after she was bitten by a bat. Treatment included induction of coma while a native...

EDITORIAL: Mild Cognitive Impairment â No Benefit from Vitamin E, Little from Donepezil

Deborah Blacker, M.D., Sc.D. — Thu, 09Â JunÂ 2005 00:00:00 -05:00

In this issue of the Journal, Petersen et al. present the long-awaited results of a randomized, placebo-controlled trial of donepezil, a standard therapy for Alzheimer's disease, and the widely used...

REVIEW ARTICLE: Calpains and Disease

Mayana Zatz, Ph.D., and Alessandra Starling, Ph.D. — Thu, 09Â JunÂ 2005 00:00:00 -05:00

Calpains are Ca2+-dependent cysteine proteases (proteolytic enzymes with cysteine in the catalytic site) that modulate cellular function. In humans, 14 independent genes encode members of the calpain superfamily. Some calpain...

ORIGINAL ARTICLE: Vitamin E and Donepezil for the Treatment of Mild Cognitive Impairment

Thu, 09Â JunÂ 2005 00:00:00 -05:00

Background: Mild cognitive impairment is a transitional state between the cognitive changes of normal aging and early Alzheimer's disease.

Methods: In a double-blind study, we evaluated subjects with the amnestic subtype...

ORIGINAL ARTICLE: A Vaccine to Prevent Herpes Zoster and Postherpetic Neuralgia in Older Adults

Thu, 02Â JunÂ 2005 00:00:00 -05:00

Background: The incidence and severity of herpes zoster and postherpetic neuralgia increase with age in association with a progressive decline in cell-mediated immunity to varicellaâzoster virus (VZV). We tested the...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 16-2005 â A Nine-Year-Old Girl with Headaches and Hypertension

Thu, 26Â MayÂ 2005 00:00:00 -05:00

A nine-year-old girl was admitted to this hospital because of headaches, enuresis, recent visual changes, and vomiting.

Intermittent frontal headaches began 18 months before admission, occurring most often during physical activity,...

EDITORIAL: Early Use of Drastic Therapy

Kenneth I. Weinberg, M.D. — Thu, 19Â MayÂ 2005 00:00:00 -05:00

Krabbe's disease, or globoid-cell leukodystrophy, is an inborn error of lipid metabolism first described in 1916 in two children with spasticity who died in infancy and were found to have...

ORIGINAL ARTICLE: Transplantation of Umbilical-Cord Blood in Babies with Infantile Krabbe's Disease

Thu, 19Â MayÂ 2005 00:00:00 -05:00

Background: Infantile Krabbe's disease produces progressive neurologic deterioration and death in early childhood. We hypothesized that transplantation of umbilical-cord blood from unrelated donors before the development of symptoms would favorably...

PERSPECTIVE: Traumatic Brain Injury in the War Zone

Susan Okie, M.D. — Thu, 19Â MayÂ 2005 00:00:00 -05:00

Sergeant David Emme, a supply officer with a U.S. Army Stryker Brigade, was stationed at a submachine gun on a truck rolling through northern Iraq last November, in a convoy...

IMAGES IN CLINICAL MEDICINE: Wernicke's Encephalopathy

Brian Kaineg, M.D. and Patricia A. Hudgins, M.D. — Thu, 12Â MayÂ 2005 00:00:00 -05:00

A 21-year-old woman with schizoaffective disorder and a four-month history of diffuse abdominal pain, nausea, vomiting, and weight loss of more than 13 kg was admitted to the hospital because...

ORIGINAL ARTICLE: Autoantibodies to Folate Receptors in the Cerebral Folate Deficiency Syndrome

Thu, 12Â MayÂ 2005 00:00:00 -05:00

In infantile-onset cerebral folate deficiency, 5-methyltetrahydrofolate (5MTHF) levels in the cerebrospinal fluid are low, but folate levels in the serum and erythrocytes are normal. We examined serum specimens from 28...

PERSPECTIVE: Autoimmune Folate Deficiency and the Rise and Fall of âHorror Autotoxicusâ

Robert S. Schwartz, M.D. — Thu, 12Â MayÂ 2005 00:00:00 -05:00

In 1904, a little more than a century ago, Julius Donath, a senior physician working in the Vienna Merchants' Hospital, and Karl Landsteiner, the chair of the pathology department at...

IMAGES IN CLINICAL MEDICINE: Painless Loss of Vision after Vomiting

Grace S. Zhang, M.D. and Jeevan R. Mathura, Jr., M.D. — Thu, 28Â AprÂ 2005 00:00:00 -05:00

A 24-year-old woman (gravida 3, para 2) who was 30 weeks' pregnant with twins was admitted for preterm labor. Six days earlier, she had vomited and noticed an acute onset...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 13-2005 â A 48-Year-Old Man with Weakness of the Limbs and Multiple Tumors of Spinal Nerves

Thu, 28Â AprÂ 2005 00:00:00 -05:00

A 48-year-old, right-handed man was evaluated in the neurology clinic of this hospital because of weakness of his arms and legs.

When the patient was 18 years of age, a mass...

REVIEW ARTICLE: Thrombosis of the Cerebral Veins and Sinuses

Jan Stam, M.D., Ph.D. — Thu, 28Â AprÂ 2005 00:00:00 -05:00

05042828.xml Thrombosis of the cerebral veins and sinuses is a distinct cerebrovascular disorder that, unlike arterial stroke, most often affects young adults and children. The symptoms and clinical course...

CLINICAL PRACTICE: Rehabilitation after Stroke

Bruce H. Dobkin, M.D. — Thu, 21Â AprÂ 2005 00:00:00 -05:00

A 66-year-old man was suddenly unable to speak, follow directions, or move his right arm and leg. He received tissue plasminogen activator within 90 minutes. Four days later, his speech...

ORIGINAL ARTICLE: Modification of Human Hearing Loss by Plasma-Membrane Calcium Pump PMCA2

Thu, 14Â AprÂ 2005 00:00:00 -05:00

Five adult siblings presented with autosomal recessive sensorineural hearing loss: two had high-frequency loss, whereas the other three had severe-to-profound loss affecting all frequencies. Genetic evaluation revealed that a homozygous...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Amyotrophic Lateral Sclerosis â A New Role for Old Drugs

Robert H. Brown, Jr., D.Phil., M.D. — Thu, 31Â MarÂ 2005 00:00:00 -05:00

Over the past decade, there have been extraordinary advances in understanding the molecular physiology of the brain and its disorders. Unfortunately, few if any of these insights have been translated...

EDITORIAL: Combination Therapy for Neuropathic Pain â Which Drugs, Which Combination, Which Patients?

Srinivasa N. Raja, M.D., and Jennifer A. Haythornthwaite, Ph.D. — Thu, 31Â MarÂ 2005 00:00:00 -05:00

Neuropathic pain is a complex, costly condition resulting from a primary lesion or dysfunction in any part of the nervous system, from the peripheral receptor to the brain. Degenerative spine...

EDITORIAL: Warfarin, Aspirin, and Intracranial Vascular Disease

Walter J. Koroshetz, M.D. — Thu, 31Â MarÂ 2005 00:00:00 -05:00

The WarfarinâAspirin Symptomatic Intracranial Disease (WASID) Trial, funded by the National Institute of Neurological Disorders and Stroke and reported by Chimowitz et al.1 in this issue of the Journal, was...

REVIEW ARTICLE: Chronic Inflammatory Demyelinating Polyneuropathy

Thu, 31Â MarÂ 2005 00:00:00 -05:00

Chronic inflammatory demyelinating polyneuropathy is a common, albeit underdiagnosed, and potentially treatable disease with an estimated prevalence of about 0.5 per 100,000 children1 and 1 to 2 per 100,000 adults.23...

ORIGINAL ARTICLE: Morphine, Gabapentin, or Their Combination for Neuropathic Pain

Thu, 31Â MarÂ 2005 00:00:00 -05:00

Background: The available drugs to treat neuropathic pain have incomplete efficacy and dose-limiting adverse effects. We compared the efficacy of a combination of gabapentin and morphine with that of each...

ORIGINAL ARTICLE: Comparison of Warfarin and Aspirin for Symptomatic Intracranial Arterial Stenosis

Thu, 31Â MarÂ 2005 00:00:00 -05:00

Background: Atherosclerotic intracranial arterial stenosis is an important cause of stroke. Warfarin is commonly used in preference to aspirin for this disorder, but these therapies have not been compared in...

IMAGES IN CLINICAL MEDICINE: Severe Fecal Impaction

Yann LoubiÃ¨res, M.D. and Olivier Chereau, M.D. — Thu, 24Â MarÂ 2005 00:00:00 -05:00

A 49-year-old man with a history of paraplegia due to poliomyelitis, surgical treatment of a lumbar ependymoma, and chronic renal insufficiency due to a neuropathic bladder was admitted to the...

REVIEW ARTICLE: The Serotonin Syndrome

Edward W. Boyer, M.D., Ph.D., and Michael Shannon, M.D., M.P.H. — Thu, 17Â MarÂ 2005 00:00:00 -05:00

The serotonin syndrome is a potentially life-threatening adverse drug reaction that results from therapeutic drug use, intentional self-poisoning, or inadvertent interactions between drugs. Three features of the serotonin syndrome are...

ORIGINAL ARTICLE: Gene Silencing and Benefit from Temozolomide in Glioblastoma

Thu, 10Â MarÂ 2005 00:00:00 -05:00

Background: Epigenetic silencing of the MGMT (O6-methylguanineâDNA methyltransferase) DNA-repair gene by promoter methylation compromises DNA repair and has been associated with longer survival in patients with glioblastoma who receive alkylating...

ORIGINAL ARTICLE: Radiotherapy plus Concomitant and Adjuvant Temozolomide for Glioblastoma

Thu, 10Â MarÂ 2005 00:00:00 -05:00

Background: Glioblastoma, the most common primary brain tumor in adults, is usually rapidly fatal. The current standard of care for newly diagnosed glioblastoma is surgical resection to the extent feasible,...

ORIGINAL ARTICLE: Treatment of Early Childhood Medulloblastoma by Postoperative Chemotherapy Alone

Thu, 10Â MarÂ 2005 00:00:00 -05:00

Background: The prognosis for young children with medulloblastoma is poor, and survivors are at high risk for cognitive deficits. We conducted a trial of the treatment of this brain tumor...

EDITORIAL: Chemotherapy for Brain Tumors â A New Beginning

Lisa M. DeAngelis, M.D. — Thu, 10Â MarÂ 2005 00:00:00 -05:00

Primary brain tumors often resist treatment; the most common brain tumor in adults, glioblastoma, kills patients within a median of a year after diagnosis, even with aggressive surgical resection and...

CLINICAL PRACTICE: Neurocardiogenic Syncope

Blair P. Grubb, M.D. — Thu, 10Â MarÂ 2005 00:00:00 -05:00

A 23-year-old nurse presents for evaluation after having had five episodes of syncope at work during the previous three months. All the episodes occurred while she was standing and were...

CLINICAL PROBLEM-SOLVING: On the Threshold â A Diagnosis of Exclusion

Peter Clarke, M.D., Susan Glick, M.D., and Brendan M. Reilly, M.D. — Thu, 03Â MarÂ 2005 00:00:00 -05:00

A 48-year-old airline mechanic from Belize presented to the emergency department with fever and altered mental status. Two weeks earlier, fever, myalgias, and dry cough had developed. His maximal daily...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 6-2005 â A 58-Year-Old Man with Esophageal Cancer and Nausea, Vomiting, and Intractable Hiccups

Thu, 24Â FebÂ 2005 00:00:00 -05:00

Dr. Kevin R. McDonald: A 58-year-old man was transferred to this hospital for management of newly diagnosed adenocarcinoma of the esophagus.

Several months before admission, the patient initially had episodes of...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Attacking Amyloid

Jeffery W. Kelly, Ph.D. — Thu, 17Â FebÂ 2005 00:00:00 -05:00

Protein aggregation seems to cause numerous neurodegenerative diseases, such as Alzheimer's disease, Parkinson's disease, and familial amyloid polyneuropathy and cardiomyopathy. Once initiated, the aggregation of individual proteins into amyloid fibrils...

REVIEW ARTICLE: Memory Dysfunction

Andrew E. Budson, M.D., and Bruce H. Price, M.D. — Thu, 17Â FebÂ 2005 00:00:00 -05:00

Memory function is vulnerable to a variety of pathologic processes including neurodegenerative diseases, strokes, tumors, head trauma, hypoxia, cardiac surgery, malnutrition, attention-deficit disorder, depression, anxiety, the side effects of medication,...

EDITORIAL: Deep-Brain Stimulation for Generalized Dystonia

Paul Greene, M.D. — Thu, 03Â FebÂ 2005 00:00:00 -05:00

Dystonia is characterized by twisting postures, tremor, and pain resulting from sustained muscle contractions. Idiopathic dystonia, in which dystonia is the only neurologic symptom, follows a very different course, depending...

ORIGINAL ARTICLE: Bilateral Deep-Brain Stimulation of the Globus Pallidus in Primary Generalized Dystonia

Thu, 03Â FebÂ 2005 00:00:00 -05:00

Background: Severe forms of dystonia respond poorly to medical treatment. Deep-brain stimulation is a reversible neurosurgical procedure that has been used for the treatment of dystonia, but assessment of its...

EDITORIAL: Early Vascular Risk Factor Modification in Type 1 Diabetes

Bruce A. Perkins, M.D., M.P.H., and Vera Bril, M.D. — Thu, 27Â JanÂ 2005 00:00:00 -05:00

The discovery of insulin some 80 years ago changed type 1 diabetes from an imminently lethal disease to a chronic condition, and clinical care evolved from preventing death to preventing...

ORIGINAL ARTICLE: Vascular Risk Factors and Diabetic Neuropathy

Thu, 27Â JanÂ 2005 00:00:00 -05:00

Background: Other than glycemic control, there are no treatments for diabetic neuropathy. Thus, identifying potentially modifiable risk factors for neuropathy is crucial. We studied risk factors for the development of...

EDITORIAL: Alcohol Consumption and Cognition

Denis A. Evans, M.D., and Julia L. Bienias, Sc.D. — Thu, 20Â JanÂ 2005 00:00:00 -05:00

Loss of cognitive function in old age, especially severe cognitive loss due to Alzheimer's disease, is a serious public health problem that will only increase as the number of people...

ORIGINAL ARTICLE: Effects of Moderate Alcohol Consumption on Cognitive Function in Women

Thu, 20Â JanÂ 2005 00:00:00 -05:00

Background: The adverse effects of excess alcohol intake on cognitive function are well established, but the effect of moderate consumption is uncertain.

Methods: Between 1995 and 2001, we evaluated cognitive function...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 1-2005 â A 35-Year-Old Woman with Difficulty Walking, Headache, and Nausea

Thu, 13Â JanÂ 2005 00:00:00 -05:00

A 35-year-old woman was admitted to the emergency department because of difficulty walking, headache, and nausea.

Two weeks before admission, the patient had noticed that her gait had become increasingly unbalanced,...

ORIGINAL ARTICLE: The Risk of Hemorrhage after Radiosurgery for Cerebral Arteriovenous Malformations

Thu, 13Â JanÂ 2005 00:00:00 -05:00

Background: Angiography shows that stereotactic radiosurgery obliterates most cerebral arteriovenous malformations after a latency period of a few years. However, the effect of this procedure on the risk of hemorrhage...

ORIGINAL ARTICLE: Mild Intraoperative Hypothermia during Surgery for Intracranial Aneurysm

Thu, 13Â JanÂ 2005 00:00:00 -05:00

Background: Surgery for intracranial aneurysm often results in postoperative neurologic deficits. We conducted a randomized trial at 30 centers to determine whether intraoperative cooling during open craniotomy would improve the...

PERSPECTIVE: Ruptured Cerebral Aneurysms

Dilantha B. Ellegala, M.D., and Arthur L. Day, M.D. — Thu, 13Â JanÂ 2005 00:00:00 -05:00

The brain derives its blood supply from two internal carotid arteries, which supply most of the cerebrum, and two vertebral arteries, which merge to form the basilar artery and supply...

PERSPECTIVE: Tales from the Temporal Lobes

Adam Zeman, D.M. — Thu, 13Â JanÂ 2005 00:00:00 -05:00

Ten years ago, the story I was hearing did not ring any bells. It went like this. During recent months, an engineer in his 40s had been having episodes of...

ORIGINAL ARTICLE: Neurologic and Developmental Disability at Six Years of Age after Extremely Preterm Birth

Thu, 06Â JanÂ 2005 00:00:00 -05:00

Background: Birth before 26 weeks of gestation is associated with a high prevalence of neurologic and developmental disabilities in the infant during the first two years of life.

Methods: We studied...

EDITORIAL: Extreme Prematurity â The Continuing Dilemma

Betty R. Vohr, M.D., and Marilee Allen, M.D. — Thu, 06Â JanÂ 2005 00:00:00 -05:00

The tremendous advances in perinatal and neonatal care that were made beginning in the 1970s and through the 1990s have contributed to the survival of infants as immature as 22...

EDITORIAL: A New Treatment for Ocular Neovascularization

Frederick L. Ferris III, M.D. — Thu, 30Â DecÂ 2004 00:00:00 -05:00

Neovascularization leads to blindness in a large number of ocular disorders, most notably age-related macular degeneration, diabetic retinopathy, retinopathy of prematurity, and other vascular occlusive diseases. Various versions of scatter-photocoagulation...

ORIGINAL ARTICLE: Pegaptanib for Neovascular Age-Related Macular Degeneration

Thu, 30Â DecÂ 2004 00:00:00 -05:00

Background: Pegaptanib, an antiâvascular endothelial growth factor therapy, was evaluated in the treatment of neovascular age-related macular degeneration.

Methods: We conducted two concurrent, prospective, randomized, double-blind, multicenter, dose-ranging, controlled clinical trials...

IMAGES IN CLINICAL MEDICINE: The Tale of Phineas Gage, Digitally Remastered

Peter Ratiu, M.D. and Ion-Florin Talos, M.D. — Thu, 02Â DecÂ 2004 00:00:00 -05:00

On September 13, 1848, while using a tamping iron to pack explosive into a rock, Phineas Gage, a 25-year-old construction foreman, triggered an uncontrolled explosion that propelled the tool (which...

IMAGES IN CLINICAL MEDICINE: Left Nasal Discharge and Meningitis

Gregory Youngnam Chang, M.D. — Thu, 18Â NovÂ 2004 00:00:00 -05:00

A 29-year-old soldier had a two-day history of headache and fever. He reported having had an intermittent, clear nasal discharge from the left nostril since his involvement in a minor...

ORIGINAL ARTICLE: Mutant Mitochondrial Elongation Factor G1 and Combined Oxidative Phosphorylation Deficiency

Thu, 11Â NovÂ 2004 00:00:00 -05:00

Although most components of the mitochondrial translation apparatus are encoded by nuclear genes, all known molecular defects associated with impaired mitochondrial translation are due to mutations in mitochondrial DNA. We...

REVIEW ARTICLE: Neonatal Brain Injury

Donna M. Ferriero, M.D. — Thu, 04Â NovÂ 2004 00:00:00 -05:00

The mortality from acute neurologic disorders of childhood, such as status epilepticus and stroke, is highest in infants under one year of age.12 Certain forms of newborn brain injury, such...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 33-2004 â A 34-Year-Old Man with a Seizure and a Frontal-Lobe Brain Lesion

Thu, 28Â OctÂ 2004 00:00:00 -05:00

A 34-year-old man was admitted to the hospital because of a seizure and a lesion in the frontal lobe of the brain.

The patient had a history of obstructive sleep apnea...

ORIGINAL ARTICLE: Clinical Features and Prognostic Factors in Adults with Bacterial Meningitis

Thu, 28Â OctÂ 2004 00:00:00 -05:00

Background: We conducted a nationwide study in the Netherlands to determine clinical features and prognostic factors in adults with community-acquired acute bacterial meningitis.

Methods: From October 1998 to April 2002, all...

PERSPECTIVE: Bacterial Meningitis â A View of the Past 90 Years

Morton N. Swartz, M.D. — Thu, 28Â OctÂ 2004 00:00:00 -05:00

The history of community-acquired bacterial meningitis arguably represents the best example of the salutary effect of the introduction of antimicrobial agents. Before the use of specific antiserums, the outlook for...

EDITORIAL: Adjunctive Steroids for Tuberculous Meningitis â More Evidence, More Questions

Vincent Quagliarello, M.D. — Thu, 21Â OctÂ 2004 00:00:00 -05:00

Tuberculosis continues to plague global health. The World Health Organization (WHO) has estimated an annual incidence of 8.8 million cases of tuberculosis worldwide, with 1.8 million deaths. Most of the...

ORIGINAL ARTICLE: Dexamethasone for the Treatment of Tuberculous Meningitis in Adolescents and Adults

Thu, 21Â OctÂ 2004 00:00:00 -05:00

Background: Tuberculous meningitis kills or disables more than half of those affected with the disease. Previous studies have been too small to determine whether adjunctive treatment with corticosteroids can reduce...

PERSPECTIVE: Tuberculous Meningitis

Peter R. Donald, M.D., and Johan F. Schoeman, M.D. — Thu, 21Â OctÂ 2004 00:00:00 -05:00

As is vividly illustrated by the report by Thwaites et al. in this issue of the Journal (pages 1741â1751), tuberculous meningitis continues to exact a devastating toll in developed and...

IMAGES IN CLINICAL MEDICINE: Communicating Hydrocephalus

J.P. Newman, Ph.D. and Ricardo Segal, M.D. — Thu, 30Â SepÂ 2004 00:00:00 -05:00

A 56-year-old woman, who was a pediatric surgeon, presented with a five-year history of memory deficits, loss of professional self-confidence, and dysphoric mood that was unresponsive to antidepressant medication; her...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 30-2004 â A 37-Year-Old Woman with Paresthesias of the Arms and Legs

Peter W. Marks, M.D., Ph.D., and Lawrence R. Zukerberg, M.D. — Thu, 23Â SepÂ 2004 00:00:00 -05:00

A 37-year-old woman was seen in the clinic because of numbness in the arms and legs.

Four months earlier, she had noticed tingling and numbness in the fingertips, without weakness, three...

CLINICAL PRACTICE: Bell's Palsy

Donald H. Gilden, M.D. — Thu, 23Â SepÂ 2004 00:00:00 -05:00

A healthy 50-year-old man notices that his face is drooping on the right side. On examination, facial asymmetry is evident, and some saliva has accumulated on the right side of...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Artificial Sweeteners â Enhancing Glycosylation to Treat Muscular Dystrophies

Thomas A. Rando, M.D., Ph.D. — Thu, 16Â SepÂ 2004 00:00:00 -05:00

A large number of genetic mutations that cause muscular dystrophies have been identified. Most forms of muscular dystrophy are caused by mutations that result in protein deficiencies. This knowledge has...

IMAGES IN CLINICAL MEDICINE: MarchiafavaâBignami Disease

Philippe Demaerel, M.D., Ph.D. and Wim Van Paesschen, M.D., Ph.D. — Thu, 09Â SepÂ 2004 00:00:00 -05:00

A 45-year-old man with chronic alcoholism was found at home with altered consciousness. On admission, neurologic examination showed disorientation, perseveration, dysarthria, dyspraxia, peripheral sensory neuropathy, and coarse rigidity of all...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 27-2004 â A 79-Year-Old Woman with Disturbances in Gait, Cognition, and Autonomic Function

Thu, 26Â AugÂ 2004 00:00:00 -05:00

A 79-year-old woman was evaluated in a neurology clinic because of difficulty walking and cognitive changes.

The patient had been well until 18 months earlier, when she began to fall occasionally....

REVIEW ARTICLE: Flavivirus Encephalitis

Tom Solomon, M.D., Ph.D. — Thu, 22Â JulÂ 2004 00:00:00 -05:00

During the summers of 2002 and 2003, North America was affected by its largest-ever outbreaks of arboviral encephalitis. West Nile virus caused 2942 cases of meningitis or encephalitis in 2002,...

ORIGINAL ARTICLE: Methylprednisolone, Valacyclovir, or the Combination for Vestibular Neuritis

Thu, 22Â JulÂ 2004 00:00:00 -05:00

Background: Vestibular neuritis is the second most common cause of peripheral vestibular vertigo. Its assumed cause is a reactivation of herpes simplex virus type 1 infection. Therefore, corticosteroids, antiviral agents,...

PERSPECTIVE: Vestibular Neuritis, or Driving Dizzily through Donegal

Richard T. Johnson, M.D. — Thu, 22Â JulÂ 2004 00:00:00 -05:00

Ten years ago, while touring the west coast of Ireland, I had a sudden onset of vertigo without hearing loss, tinnitus, or fullness in an ear. As a neurologist, I...

IMAGES IN CLINICAL MEDICINE: A Medical Mystery â Which Twin Is the Patient?

Willy-Anne Nieuwlaat, M.D. and Gerlach Pieters, M.D., Ph.D. — Thu, 01Â JulÂ 2004 00:00:00 -05:00

Which of these 50-year-old identical twins is the patient? And what is the diagnosis?...

REVIEW ARTICLE: Alzheimer's Disease

Jeffrey L. Cummings, M.D. — Thu, 01Â JulÂ 2004 00:00:00 -05:00

Alzheimer's disease is a progressive and fatal neurodegenerative disorder manifested by cognitive and memory deterioration, progressive impairment of activities of daily living, and a variety of neuropsychiatric symptoms and behavioral...

CLINICAL PRACTICE: Neuropathic Diabetic Foot Ulcers

Andrew J.M. Boulton, M.D., Robert S. Kirsner, M.D., and Loretta Vileikyte, M.D. — Thu, 01Â JulÂ 2004 00:00:00 -05:00

A 58-year-old man with type 2 diabetes mellitus has an asymptomatic plantar ulcer on the left foot that remains unhealed after four months. The ulcer measures 2 cm by 1...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Erythropoietin for Neurologic Protection and Diabetic Neuropathy

Stuart A. Lipton, M.D., Ph.D. — Thu, 10Â JunÂ 2004 00:00:00 -05:00

The majority of drugs developed for neurologic indications fail because they are not clinically tolerated. A sensible strategy, therefore, is to explore the efficacy of drugs in the nervous system...

IMAGES IN CLINICAL MEDICINE: Cicatricial Pemphigoid

Conleth A. Egan, M.R.C.P.I. and Scott R. Florell, M.D. — Thu, 10Â JunÂ 2004 00:00:00 -05:00

A 26-year-old man presented with blisters and erosions that had developed on his tongue over a 48-hour period (Panel A). The blisters had appeared primarily in his mouth, but several...

ORIGINAL ARTICLE: Tumors of the Endolymphatic Sac in von HippelâLindau Disease

Thu, 10Â JunÂ 2004 00:00:00 -05:00

Tumors of the endolymphatic sac are locally invasive neoplasms arising in the temporal bone that can cause hearing loss, tinnitus, vertigo, aural fullness, and facial-nerve dysfunction. They occur sporadically but...

REVIEW ARTICLE: Diverse Causes of Hypoglycemia-Associated Autonomic Failure in Diabetes

Philip E. Cryer, M.D. — Thu, 27Â MayÂ 2004 00:00:00 -05:00

Iatrogenic hypoglycemia is the limiting factor in the glycemic management of diabetes.12 It causes recurrent symptomatic and sometimes, at least temporarily, disabling episodes in most people with type 1 diabetes...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 16-2004 â A 76-Year-Old Woman with Numbness and Pain in the Feet and Legs

Anthony A. Amato, M.D., and Anne Louise Oaklander, M.D., Ph.D. — Thu, 20Â MayÂ 2004 00:00:00 -05:00

A 76-year-old woman was referred to the Pain Center of this hospital because of pain and numbness in her feet and legs. Six years earlier, intermittent numbness had developed in...

IMAGES IN CLINICAL MEDICINE: Papilledema and Spinal Cord Tumor

Seyed F.A. Amlashi, M.D. and Laurent Riffaud, M.D. — Thu, 13Â MayÂ 2004 00:00:00 -05:00

A 49-year-old man presented with a three-week history of progressive visual loss and headaches. Funduscopic examination revealed bilateral papilledema. Computed tomographic examination of the cerebrum showed a moderate-sized, communicating hydrocephalus....

IMAGES IN CLINICAL MEDICINE: Stretch Brachial Plexopathy

Judith Lynn Gorelick, M.D. — Thu, 13Â MayÂ 2004 00:00:00 -05:00

A 29-year-old man had a one-month history of weakness of the left hand and painful paresthesias involving the radial half of his palm, as well as the palmar aspect of...

ORIGINAL ARTICLE: Cord-Blood Transplants from Unrelated Donors in Patients with Hurler's Syndrome

Thu, 06Â MayÂ 2004 00:00:00 -05:00

Background: Hurler's syndrome (the most severe form of mucopolysaccharidosis type I) causes progressive deterioration of the central nervous system and death in childhood. Allogeneic bone marrow transplantation before the age...

PERSPECTIVE: Advances in the Treatment of Mucopolysaccharidosis Type I

Joseph Muenzer, M.D., Ph.D., and Amy Fisher, M.S. — Thu, 06Â MayÂ 2004 00:00:00 -05:00

The mucopolysaccharidoses are a group of lysosomal storage diseases caused by a deficiency of enzymes that degrade glycosaminoglycans.1 Mucopolysaccharidosis type I, an autosomal recessive disorder caused by a deficiency of...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 14-2004 â A 66-Year-Old Man with Progressive Neurologic Deficits

Thu, 29Â AprÂ 2004 00:00:00 -05:00

A 66-year-old, right-handed man was admitted to the hospital because of progressive left-sided weakness.

Nineteen years before admission, the patient had received a renal transplant from a living related donor because...

IMAGES IN CLINICAL MEDICINE: The Mount Fuji Sign

Josef G. Heckmann, M.D. and Oliver Ganslandt, M.D. — Thu, 29Â AprÂ 2004 00:00:00 -05:00

A 53-year-old woman was admitted because of progressive right-sided hemiparesis and aphasia. A computed tomographic (CT) scan of the brain revealed a space-occupying supratentorial lesion. A biopsy through a burr...

IMAGES IN CLINICAL MEDICINE: Hemispheric Arteriovenous Malformation

John Mark Vermillion, M.D. and F.S. Harris, M.D. — Thu, 22Â AprÂ 2004 00:00:00 -05:00

Evaluation of a 23-year-old man with a history of tonicâclonic seizures revealed an arteriovenous malformation in the right parietal region of the brain (Panel A, arrow). Angiographic study of the...

IMAGES IN CLINICAL MEDICINE: Angiomatosis Associated with Sneddon's Syndrome

Josef G. Heckmann, M.D. and M. LÃ¼fti, M.D. — Thu, 18Â MarÂ 2004 00:00:00 -05:00

A 34-year-old woman was admitted for acute right hemiparesis, which resolved within 24 hours. She had no history of hypertension, diabetes, migraine, or smoking. Since early childhood, she had had...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 9-2004 â An 18-Year-Old Man with Respiratory Symptoms and Shock

Thu, 18Â MarÂ 2004 00:00:00 -05:00

Dr. I. David Todres (Pediatric Intensive Care): An eighteen-year-old man was admitted to the hospital in shock after a five-day illness.

The patient had been in good health until five...

ORIGINAL ARTICLE: Calcitonin GeneâRelated Peptide Receptor Antagonist BIBN 4096 BS for the Acute Treatment of Migraine

Thu, 11Â MarÂ 2004 00:00:00 -05:00

Background: Calcitonin geneârelated peptide (CGRP) may have a causative role in migraine. We therefore hypothesized that a CGRP-receptor antagonist might be effective in the treatment of migraine attacks.

Methods: In an...

PERSPECTIVE: CGRP-Receptor Antagonists â A Fresh Approach to Migraine Therapy?

Paul L. Durham, Ph.D. — Thu, 11Â MarÂ 2004 00:00:00 -05:00

Migraine is a chronic disease that affects 12 percent of the general population. In addition to severe, debilitating headache, patients with migraines may experience nausea, vomiting, and sensitivity to light...

ORIGINAL ARTICLE: Use of the Inactivated Intranasal Influenza Vaccine and the Risk of Bell's Palsy in Switzerland

Thu, 26Â FebÂ 2004 00:00:00 -05:00

Background: After the introduction of an inactivated intranasal influenza vaccine that was used only in Switzerland, 46 cases of Bell's palsy were reported.

Methods: We conducted a matched caseâcontrol study and...

PERSPECTIVE: Nasal Vaccination, Enterotoxin, and Bell's Palsy

Robert B. Couch, M.D. — Thu, 26Â FebÂ 2004 00:00:00 -05:00

In 1997, a Swiss Company (Berna Biotech) received approval to market an inactivated influenza vaccine for parenteral administration that consists of the hemagglutinin and neuraminidase surface antigens of influenzavirus incorporated...

REVIEW ARTICLE: Acute Chemical Emergencies

Stefanos N. Kales, M.D., M.P.H., and David C. Christiani, M.D., M.P.H. — Thu, 19Â FebÂ 2004 00:00:00 -05:00

Acute chemical emergencies can occur as a result of an industrial disaster,12 occupational exposure,34 recreational mishap,5 natural catastrophe,6 chemical warfare,78 and acts of terrorism.910 This article reviews the health effects...

REVIEW ARTICLE: Management of Overactive Bladder

Joseph G. Ouslander, M.D. — Thu, 19Â FebÂ 2004 00:00:00 -05:00

Overactive bladder is a symptom complex that includes urinary urgency with or without urge incontinence, urinary frequency (voiding eight or more times in a 24-hour period), and nocturia (awakening two...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 5-2004 â A 57-Year-Old Man with Slurred Speech and Left Hemiparesis

Thu, 12Â FebÂ 2004 00:00:00 -05:00

A 57-year-old right-handed man was admitted to the hospital because of the sudden onset of slurred speech and left hemiparesis.

The patient had been well until two days earlier, when he...

IMAGES IN CLINICAL MEDICINE: Hypoglossal-Nerve Palsy

Dimitrios I. Zafeiriou,, M.D., Ph.D. and Evangelos Pavlou, M.D. — Thu, 29Â JanÂ 2004 00:00:00 -05:00

A six-year-old boy was brought to the emergency department because of acute speech difficulties. Five days previously, a low-grade fever, sore throat, and malaise had developed. The day before presentation,...

EDITORIAL: Neurocysticercosis â Is the Elimination of Parasites Beneficial?

Julio Sotelo, M.D. — Thu, 15Â JanÂ 2004 00:00:00 -05:00

Taenia solium causes two different diseases. When the adult cestode infests the human intestine, taeniasis develops; it is generally asymptomatic, but the host becomes a continuous source of taenia eggs,...

IMAGES IN CLINICAL MEDICINE: Neurocysticercosis

Howard W. Sander, M.D. and Conrado Castro, M.D. — Thu, 15Â JanÂ 2004 00:00:00 -05:00

A 15-year-old Peruvian girl who lived in the Andes mountains had a three-month history of headache, nausea, vomiting, and visual obscuration and a one-month history of incoherent speech, confusion, and...

ORIGINAL ARTICLE: A Trial of Antiparasitic Treatment to Reduce the Rate of Seizures Due to Cerebral Cysticercosis

Thu, 15Â JanÂ 2004 00:00:00 -05:00

Background: Neurocysticercosis is the main cause of adult-onset seizures in the developing world. Whether therapy with antiparasitic agents results in improved seizure control has been questioned because of the lack...

PERSPECTIVE: Tapeworms and Seizures â Treatment and Prevention

James H. Maguire, M.D. — Thu, 15Â JanÂ 2004 00:00:00 -05:00

It may come as a surprise that a lowly tapeworm is responsible for as many as 10 percent of cases in which a patient presents with seizures to an emergency...

IMAGES IN CLINICAL MEDICINE: Optic Neuritis Due to Infection

Arnd M. Herz, M.D. and J. Michael Lahey, M.D. — Thu, 08Â JanÂ 2004 00:00:00 -05:00

A 14-year-old girl presented with worsening headaches, unilateral decreased visual acuity (20/20 in the right eye and 5/200 in the left eye), and ocular pain in the left eye. The...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 1-2004 â A 49-Year-Old Woman with Asymmetric Painful Neuropathy

David A. Chad, M.D., and E. Tessa Hedley-Whyte, M.D. — Thu, 08Â JanÂ 2004 00:00:00 -05:00

A 49-year-old left-handed woman was evaluated in the neurology clinic because of painful asymmetric neuropathy.

The patient had been well until several years earlier, when numbness developed in the right hand....

REVIEW ARTICLE: Diabetic Retinopathy

Robert N. Frank, M.D. — Thu, 01Â JanÂ 2004 00:00:00 -05:00

Diabetic retinopathy is the most severe of the several ocular complications of diabetes. Advances in treatment over the past 40 years have greatly reduced the risk of blindness from this...

SOUNDING BOARD: The Meningococcal Vaccine â Public Policy and Individual Choices

Paul A. Offit, M.D., and Georges Peter, M.D. — Thu, 11Â DecÂ 2003 00:00:00 -05:00

On December 11, 2002, a 12-year-old girl from suburban Philadelphia died as a result of serogroup C meningococcal infection. Death occurred within hours after the initial manifestation of the illness....

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 38-2003 â A 12-Year-Old Girl with Fever and Coma

H. Shaw Warren, Jr., M.D., R. Gilberto Gonzalez, M.D., and Di Tian, M.D., Ph.D. — Thu, 11Â DecÂ 2003 00:00:00 -05:00

A 12-year-old girl was admitted to the hospital because of fever, vomiting, and an abrupt onset of unresponsiveness.

She had been well until one week earlier, when a cough, sore throat,...

IMAGES IN CLINICAL MEDICINE: Fabry's Disease

Jeffrey B. Kopp, M.D. and Raphael Schiffmann, M.D. — Thu, 20Â NovÂ 2003 00:00:00 -05:00

A 30-year-old man had severe neuropathic pain that affected his hands and feet. Examination of his skin showed angiokeratomas that were particularly prominent in the groin and on the back...

IMAGES IN CLINICAL MEDICINE: Pituitary Apoplexy

Leonard C. Glass, M.D. — Thu, 20Â NovÂ 2003 00:00:00 -05:00

Acute hypotension developed in a 63-year-old man during coronary-artery bypass graft surgery. On awakening from surgery, he reported a severe headache and lightheadedness. Four hours later, he was noted to...

REVIEW ARTICLE: Opioid Therapy for Chronic Pain

Jane C. Ballantyne, M.D., and Jianren Mao, M.D., Ph.D. — Thu, 13Â NovÂ 2003 00:00:00 -05:00

Opium is a bitter, brown, granular powder derived from the seedpod of the poppy (Papaver somniferum). People have used opium for the relief of pain and suffering for thousands of...

ORIGINAL ARTICLE: Extraneural Pathologic Prion Protein in Sporadic CreutzfeldtâJakob Disease

Thu, 06Â NovÂ 2003 00:00:00 -05:00

Background: In patients with sporadic CreutzfeldtâJakob disease, pathologic disease-associated prion protein (PrPSc) has been identified only in the central nervous system and olfactory-nerve tissue. Understanding the distribution of PrPSc in...

SOUNDING BOARD: Can We Prevent Cerebral Palsy?

Karin B. Nelson, M.D. — Thu, 30Â OctÂ 2003 00:00:00 -05:00

Improvements in obstetrical and neonatal care have led to marked declines in rates of injury and death in mothers and infants. The risk of neurologic disability in infants and children...

REVIEW ARTICLE: Schizophrenia

Robert Freedman, M.D. — Thu, 30Â OctÂ 2003 00:00:00 -05:00

Schizophrenia is a chronic, debilitating psychotic mental disorder that affects about 1 percent of people. A new generation of medications and recent developments in neuropathology, brain imaging, and molecular genetics...

REVIEW ARTICLE: The Toxicology of Mercury â Current Exposures and Clinical Manifestations

Thomas W. Clarkson, Ph.D., Laszlo Magos, M.D., and Gary J. Myers, M.D. — Thu, 30Â OctÂ 2003 00:00:00 -05:00

Mercury has been used commercially and medically for centuries. In the past it was a common constituent of many medications. It is still used in hospitals in thermometers and blood-pressure...

IMAGES IN CLINICAL MEDICINE: Cerebral Venous Thrombosis

Pierre-Marie Roy, M.D. and Emmanuelle Gras, M.D. — Thu, 30Â OctÂ 2003 00:00:00 -05:00

A 20-year-old, previously healthy woman presented with a progressively worsening headache. She had been well until three weeks earlier, when she started having an episodic, pulsating headache. Migraine was suspected,...

PERSPECTIVE: Tools of the Trade

Danielle Ofri, M.D., Ph.D. — Thu, 30Â OctÂ 2003 00:00:00 -05:00

I grasped the list of patients, fingering the crispness of the sheet that represented my first day of service on the medical wards. I knew that the sheet would soon...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 33-2003 â A 37-Year-Old Man with a History of Alcohol and Drug Abuse and Sudden Onset of Leg Weakness

Oscar Soto, M.D, and E. Tessa Hedley-Whyte, M.D. — Thu, 23Â OctÂ 2003 00:00:00 -05:00

A 37-year-old man was admitted to the hospital because of collapse.

The patient was unmarried and unemployed and was a former smoker. He had abused alcohol since the age of 13...

REVIEW ARTICLE: Paraneoplastic Syndromes Involving the Nervous System

Robert B. Darnell, M.D., Ph.D., and Jerome B. Posner, M.D. — Thu, 16Â OctÂ 2003 00:00:00 -05:00

The term âparaneoplastic syndromesâ refers to symptoms or signs resulting from damage to organs or tissues that are remote from the site of a malignant neoplasm or its metastases. Paraneoplastic...

REVIEW ARTICLE: Primary Progressive Aphasia â A Language-Based Dementia

M.-Marsel Mesulam, M.D. — Thu, 16Â OctÂ 2003 00:00:00 -05:00

Dementia is a generic term used to designate chronically progressive brain disease that impairs intellect and behavior to the point where customary activities of daily living become compromised.12 In some...

IMAGES IN CLINICAL MEDICINE: Epidural Lipomatosis Causing Spinal Cord Compression

Moahad Saeed Dar, M.D. and Shadi Daoud, M.D. — Thu, 09Â OctÂ 2003 00:00:00 -05:00

A 68-year-old woman with corticosteroid-dependent chronic obstructive pulmonary disease was admitted to the hospital for worsening respiratory symptoms and for new-onset weakness in both legs. Physical examination revealed poor air...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 30-2003 â A 21-Year-Old Man with Sudden Alteration of Mental Status

David N. Tancredi, M.D., and Michael W. Shannon, M.D. — Thu, 25Â SepÂ 2003 00:00:00 -05:00

Dr. Joshua N. Goldstein (Emergency Medicine): A 21-year-old man was brought to the emergency department because of a sudden change in mental status.

On the evening of admission, the patient...

CLINICAL PRACTICE: Early Alzheimer's Disease

Claudia H. Kawas, M.D. — Thu, 11Â SepÂ 2003 00:00:00 -05:00

A 72-year-old, college-educated woman comes in for the evaluation of mild memory loss that has been gradually progressing for the past two years. The patient lives alone, drives her own...

IMAGES IN CLINICAL MEDICINE: Chondroid Clival Chordoma

Andreas M. Stark, M.D. and H. Maximilian Mehdorn, M.D., Ph.D. — Thu, 04Â SepÂ 2003 00:00:00 -05:00

A 54-year-old man presented with a history of several weeks of fatigue and weight loss. He also reported atrophy of the testes and loss of secondary hair that had begun...

REVIEW ARTICLE: Drug Addiction

Drug addiction is a chronic, relapsing disorder in which compulsive drug-seeking and drug-taking behavior persists despite serious negative consequences. Addictive substances induce pleasant states (euphoria in the initiation phase) or...

REVIEW ARTICLE: Laryngology and Phonosurgery

Steven M. Zeitels, M.D., and Gerald B. Healy, M.D. — Thu, 28Â AugÂ 2003 00:00:00 -05:00

It has been more than 20 years since Vaughan1 reviewed the state of the art in human laryngology and voice disorders. During that time, an improved understanding of the physiology...

IMAGES IN CLINICAL MEDICINE: A Fetus with Hypogenesis of the Corpus Callosum

Christine M. Glastonbury, M.B., B.S. and Anne M. Kennedy, M.R.C.P. — Thu, 07Â AugÂ 2003 00:00:00 -05:00

On routine prenatal ultrasonography, the cavum septum pellucidum was absent and a possible posterior fossa malformation was noted in a 31-week male fetus with a normal karyotype. A magnetic resonance...

REVIEW ARTICLE: Molecular Mechanisms of Amyloidosis

Giampaolo Merlini, M.D., and Vittorio Bellotti, M.D., Ph.D. — Thu, 07Â AugÂ 2003 00:00:00 -05:00

The amyloidoses constitute a large group of diseases in which misfolding of extracellular protein has a prominent role. This dynamic process, which occurs in parallel with or as an alternative...

PERSPECTIVE: Cochlear Implants

George A. Gates, M.D., and Richard T. Miyamoto, M.D. — Thu, 31Â JulÂ 2003 00:00:00 -05:00

Currently, a cochlear implant is the standard treatment for people whose hearing ability is so poor that well-fit hearing aids fail to permit effective oral communication. The implant is a...

IMAGES IN CLINICAL MEDICINE: Raccoon Eyes and Neuroblastoma

Robert Timmerman, M.D. — Thu, 24Â JulÂ 2003 00:00:00 -05:00

A nine-month-old girl was seen for failure to thrive and bruising around the eyes. On physical examination, she was noted to have periorbital ecchymosis, dysconjugate gaze, dilated and fixed pupils,...

PERSPECTIVE: Severe Methylenetetrahydrofolate Reductase Deficiency, Methionine Synthase, and Nitrous Oxide â A Cautionary Tale

Richard W. Erbe, M.D., and Robbert J. Salis, M.D. — Thu, 03Â JulÂ 2003 00:00:00 -05:00

In this issue of the Journal, Selzer et al. (pages 45â50) describe the unexpected neurologic deterioration and death of an infant boy who had been anesthetized twice within a short...

ORIGINAL ARTICLE: Adverse Effect of Nitrous Oxide in a Child with 5,10-Methylenetetrahydrofolate Reductase Deficiency

Thu, 03Â JulÂ 2003 00:00:00 -05:00

Nitrous oxide irreversibly oxidizes the cobalt atom of vitamin B12, thereby inhibiting the activity of the cobalamin-dependent enzyme methionine synthase (or 5-methyltetrahydrofolateâhomocysteine S-methyltransferase; Enzyme Commission code EC 2.1.1.13). Methionine synthase...

REVIEW ARTICLE: Mitochondrial Respiratory-Chain Diseases

Salvatore DiMauro, M.D., and Eric A. Schon, Ph.D. — Thu, 26Â JunÂ 2003 00:00:00 -05:00

More than a billion years ago, aerobic bacteria colonized primordial eukaryotic cells that lacked the ability to use oxygen metabolically. A symbiotic relationship developed and became permanent. The bacteria evolved...

ORIGINAL ARTICLE: Leisure Activities and the Risk of Dementia in the Elderly

Thu, 19Â JunÂ 2003 00:00:00 -05:00

Background: Participation in leisure activities has been associated with a lower risk of dementia. It is unclear whether increased participation in leisure activities lowers the risk of dementia or participation...

PERSPECTIVE: Use It or Lose It â Do Effortful Mental Activities Protect against Dementia?

Joseph T. Coyle, M.D. — Thu, 19Â JunÂ 2003 00:00:00 -05:00

The past 15 years, since the cloning of the gene for amyloid precursor protein, have witnessed breathtaking advances in our understanding of the pathophysiology of Alzheimer's disease, the most common...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 17-2003 â A 38-Year-Old Woman with Fever, Headache, and Confusion

Martin S. Hirsch, M.D., and Barbara Werner, Ph.D. — Thu, 29Â MayÂ 2003 00:00:00 -05:00

A 38-year-old woman was admitted to the hospital in early August 2002 because of fever and confusion.

The patient had been well until four days earlier, when a headache gradually developed...

IMAGES IN CLINICAL MEDICINE: Neurologic Manifestations of Vitamin B Deficiency

Katalin Scherer, M.D. — Thu, 29Â MayÂ 2003 00:00:00 -05:00

A 56-year-old woman presented with a four-month history of progressive cognitive decline, weakness, incoordination, and gait disturbance. She had a score of 12 of 28 on the MiniâMental State Examination,...

ORIGINAL ARTICLE: Pernicious Anemia with Neuropsychiatric Dysfunction in a Patient with Sickle Cell Anemia Treated with Folate Supplementation

Meekoo Dhar, M.D., Rita Bellevue, M.D., and Ralph Carmel, M.D. — Thu, 29Â MayÂ 2003 00:00:00 -05:00

03052932.xml The administration of folic acid can mask the megaloblastic anemia caused by cobalamin deficiency.12345 More critically, this masking, although neither complete nor permanent,234 can permit neurologic dysfunction to...

ORIGINAL ARTICLE: Transmission of West Nile Virus from an Organ Donor to Four Transplant Recipients

Thu, 29Â MayÂ 2003 00:00:00 -05:00

Background: In August 2002, fever and mental-status changes developed in recipients of organs from a common donor. Transmission of West Nile virus through organ transplantation was suspected.

Methods: We reviewed medical...

PERSPECTIVE: West Nile Virus â Not a Passing Phenomenon

Dale L. Morse, M.D. — Thu, 29Â MayÂ 2003 00:00:00 -05:00

As private citizens and as public health professionals, we are enthralled by the new, the unknown, and the catastrophic. This is especially true for epidemics that appear suddenly, spread rapidly,...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 16-2003 â A 58-Year-Old Woman with Left-Sided Weakness and a Right Frontal Brain Mass

Thu, 22Â MayÂ 2003 00:00:00 -05:00

A 58-year-old, right-handed woman was admitted to the hospital because of left hemiparesis.

The patient had been well until five days earlier, when a right frontal headache developed, with radiation to...

CLINICAL PRACTICE: Restless Legs Syndrome

Christopher J. Earley, M.B., B.Ch., Ph.D. — Thu, 22Â MayÂ 2003 00:00:00 -05:00

A 45-year-old woman reports having had nightly insomnia for years. On further questioning, she reports having uncomfortable sensations in her legs when she lies down at night. She describes a...

ORIGINAL ARTICLE: Intellectual Impairment in Children with Blood Lead Concentrations below 10 Î¼g per Deciliter

Thu, 17Â AprÂ 2003 00:00:00 -05:00

Background: Despite dramatic declines in children's blood lead concentrations and a lowering of the Centers for Disease Control and Prevention's level of concern to 10 Î¼g per deciliter (0.483 Î¼mol...

PERSPECTIVE: Exposure to Lead in Children â How Low Is Low Enough?

Walter J. Rogan, M.D., and James H. Ware, Ph.D. — Thu, 17Â AprÂ 2003 00:00:00 -05:00

Encephalopathy in childhood due to lead poisoning was described more than 100 years ago, and cognitive sequelae after recovery were reported in 1943. During the past three decades, epidemiologic studies...

EDITORIAL: Is Refractory Epilepsy Due to Genetically Determined Resistance to Antiepileptic Drugs?

Timothy A. Pedley, M.D., and Michio Hirano, M.D. — Thu, 10Â AprÂ 2003 00:00:00 -05:00

Failure to respond to antiseizure drugs is the usual basis for defining intractable epilepsy, although there is a lack of consensus about what constitutes drug resistance and when this determination...

ORIGINAL ARTICLE: Preimplantation Diagnosis for Sonic Hedgehog Mutation Causing Familial Holoprosencephaly

Thu, 10Â AprÂ 2003 00:00:00 -05:00

The sonic hedgehog (SHH) gene is a human homologue of the drosophila gene encoding inductive signals involved in patterning the early embryo and which is highly functionally conserved in many...

REVIEW ARTICLE: Apoptosis and Caspases in Neurodegenerative Diseases

Robert M. Friedlander, M.D. — Thu, 03Â AprÂ 2003 00:00:00 -05:00

Acute and chronic neurodegenerative diseases are illnesses associated with high morbidity and mortality, and few or no effective options are available for their treatment. A characteristic of many neurodegenerative diseases...

REVIEW ARTICLE: Alzheimer's Disease and Parkinson's Disease

Robert L. Nussbaum, M.D., and Christopher E. Ellis, Ph.D. — Thu, 03Â AprÂ 2003 00:00:00 -05:00

The incidence of many common diseases is increased among the relatives of affected patients, but the pattern of inheritance rarely follows Mendel's laws. Instead, such common diseases are thought to...

ORIGINAL ARTICLE: Memantine in Moderate-to-Severe Alzheimer's Disease

Thu, 03Â AprÂ 2003 00:00:00 -05:00

Background: Overstimulation of the N-methyl-D-aspartate (NMDA) receptor by glutamate is implicated in neurodegenerative disorders. Accordingly, we investigated memantine, an NMDA antagonist, for the treatment of Alzheimer's disease.

Methods: Patients with moderate-to-severe...

EDITORIAL: Opioids and Chronic Neuropathic Pain

Kathleen M. Foley, M.D. — Thu, 27Â MarÂ 2003 00:00:00 -05:00

Chronic neuropathic pain is a serious problem resulting from injury to the central or peripheral nervous system; it affects more than 2 million Americans. Despite advances in our understanding of...

EDITORIAL: âSilentâ Strokes and Dementia

John P. Blass, M.D., Ph.D., and Rajiv R. Ratan, M.D., Ph.D. — Thu, 27Â MarÂ 2003 00:00:00 -05:00

An epidemiologic study by Vermeer et al. in this issue of the Journal indicates that âsilentâ strokes are a risk factor for dementia.1 Recurrent strokes during the three to four...

CLINICAL PRACTICE: Painful Sensory Neuropathy

Jerry R. Mendell, M.D., and Zarife Sahenk, M.D., Ph.D. — Thu, 27Â MarÂ 2003 00:00:00 -05:00

A 67-year-old woman who had been in excellent health noticed the onset of burning pain in the left great toe two years before evaluation. The pain subsequently extended to involve...

ORIGINAL ARTICLE: Oral Opioid Therapy for Chronic Peripheral and Central Neuropathic Pain

Thu, 27Â MarÂ 2003 00:00:00 -05:00

Background: Although opioids are commonly used to treat chronic neuropathic pain, there are limited data to guide their use. Few controlled trials have been performed, and many types of neuropathic...

ORIGINAL ARTICLE: Silent Brain Infarcts and the Risk of Dementia and Cognitive Decline

Thu, 27Â MarÂ 2003 00:00:00 -05:00

Background: Silent brain infarcts are frequently seen on magnetic resonance imaging (MRI) in healthy elderly people and may be associated with dementia and cognitive decline.

Methods: We studied the association between...

CLINICAL PRACTICE: Vestibular Neuritis

Robert W. Baloh, M.D. — Thu, 13Â MarÂ 2003 00:00:00 -05:00

A 53-year-old man awoke in the morning with acute dizziness. He staggered to the bathroom, where he vomited repeatedly. When he was seen at a local emergency room 12 hours...

IMAGES IN CLINICAL MEDICINE: Fatal Cerebral Hypoxemia after Cardiac Arrest

Jesper Kelsen, M.D. and Annette Obel, M.D. — Thu, 27Â FebÂ 2003 00:00:00 -05:00

A 50-year-old woman suddenly became unconscious during her birthday celebration; there was no measurable pulse or blood pressure. Breathing and circulation returned spontaneously within a few minutes, but the exact...

EDITORIAL: Neuromuscular Sequelae of Critical Illness

Leonard D. Hudson, M.D., and Catherine M. Lee, M.D. — Thu, 20Â FebÂ 2003 00:00:00 -05:00

Until recently, survival to hospital discharge was the primary outcome considered in reports concerning populations of critically ill patients. But a body of knowledge is now being developed about longer-term...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 6-2003 â A Nine-Year-Old Girl with Progressive Weakness and Areflexia

Howard W. Sander, M.D., and E. Tessa Hedley-Whyte, M.D. — Thu, 20Â FebÂ 2003 00:00:00 -05:00

A nine-year-old right-handed girl from the United Arab Emirates was admitted to the hospital because of progressive weakness and areflexia.

The patient had been well until three months earlier, when a...

IMAGES IN CLINICAL MEDICINE: Bilateral Striatal Necrosis Associated with Infection

Miriam van Buiren, M.D. and Markus Uhl, M.D. — Thu, 20Â FebÂ 2003 00:00:00 -05:00

Fever and neutropenia developed in a 14-year-old girl after she received intensive chemotherapy for her third relapse of preâB-cell acute lymphocytic leukemia, including intrathecal therapy with methotrexate, cytarabine, and prednisone....

ORIGINAL ARTICLE: Detection of Pathologic Prion Protein in the Olfactory Epithelium in Sporadic CreutzfeldtâJakob Disease

Thu, 20Â FebÂ 2003 00:00:00 -05:00

Background: Olfactory cortexes and the olfactory tracts are involved in sporadic CreutzfeldtâJakob disease. We examined peripheral regions of the olfactory sensory pathway, including the olfactory mucosa, to assess whether pathologic...

PERSPECTIVE: CreutzfeldtâJakob Disease

Kenneth L. Tyler, M.D. — Thu, 20Â FebÂ 2003 00:00:00 -05:00

CreutzfeldtâJakob disease is an unusual neurologic disorder, with a prevalence of approximately 1 case per million population, which is about 1/10,000 that of Alzheimer's disease. CreutzfeldtâJakob disease has captured widespread...

CLINICAL PRACTICE: Preventing Falls in Elderly Persons

Mary E. Tinetti, M.D. — Thu, 02Â JanÂ 2003 00:00:00 -05:00

A 79-year-old woman with a history of congestive heart failure, arthritis, depression, and difficulty sleeping presents for a follow-up visit. She takes several prescription medications, including an antidepressant, a diuretic,...

ORIGINAL ARTICLE: Genetic, Clinical, and Radiographic Delineation of HallervordenâSpatz Syndrome

Thu, 02Â JanÂ 2003 00:00:00 -05:00

Background: HallervordenâSpatz syndrome is an autosomal recessive disorder characterized by dystonia, parkinsonism, and iron accumulation in the brain. Many patients with this disease have mutations in the gene encoding pantothenate...

ORIGINAL ARTICLE: A Controlled Trial of Natalizumab for Relapsing Multiple Sclerosis

Thu, 02Â JanÂ 2003 00:00:00 -05:00

Background: In patients with multiple sclerosis, inflammatory brain lesions appear to arise from autoimmune responses involving activated lymphocytes and monocytes. The glycoprotein Î±4 integrin is expressed on the surface of...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 39-2002 â A 35-Year-Old Man with Headache, Deviation of the Tongue, and Unusual Radiographic Abnormalities

William F.C. Rigby, Chieh-Min Fan, and Eugene J. Mark — Thu, 19Â DecÂ 2002 00:00:00 -05:00

A 35-year-old right-handed man was admitted to the hospital because of a persistent headache and deviation of the tongue.

The patient had been in excellent health until about one month earlier,...

IMAGES IN CLINICAL MEDICINE: Mycotic Aneurysms

Joshua Lynfield, M.D. and Peter Kim Nelson, M.D. — Thu, 19Â DecÂ 2002 00:00:00 -05:00

Figure 1. Headache and aphasia suddenly developed in a 49-year-old man. He had a history of aortic valvotomy for severe congenital aortic stenosis 30 years previously, and severe aortic stenosis...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Erythropoietin â An Endogenous Retinal Survival Factor

Thu, 12Â DecÂ 2002 00:00:00 -05:00

Loss of vision caused by apoptotic cell death is a feature of age-related macular degeneration, retinitis pigmentosa, glaucoma, and other degenerative retinal diseases. Degeneration of photoreceptors â cells that transduce...

IMAGES IN CLINICAL MEDICINE: Herpetic Encephalitis and Acute Retinal Necrosis

Peter W. Hadden, F.R.A.N.Z.C.O. and Christopher J. Barry, M.Med.Sci. — Thu, 12Â DecÂ 2002 00:00:00 -05:00

Figure 1. A 45-year-old man with a biopsy-proven pineal tumor was on the seventh day of his first cycle of chemotherapy when symptoms of meningeal irritation and confusion developed. Polymerase...

IMAGES IN CLINICAL MEDICINE: Tuberculous Mastoiditis and Cerebral Tuberculoma

Valerie A. Reid, M.R.C.P.I. and Joseph Keane, M.R.C.P.I. — Thu, 05Â DecÂ 2002 00:00:00 -05:00

Figure 1. A 57-year-old woman was admitted with progressive drowsiness and a change in mental status. A biopsy of an axillary node had been performed one week before admission because...

IMAGES IN CLINICAL MEDICINE: Tuberculosis with Atlantoaxial and Craniovertebral Pott's Disease

Ulrich Weber, M.D. and Hans ScherÃ¼bl, M.D. — Thu, 05Â DecÂ 2002 00:00:00 -05:00

Figure 1. A 17-year-old Ethiopian girl presented with a four-month history of fever, malaise, and weight loss, as well as head, neck, and back pain and a two-week history of...

ORIGINAL ARTICLE: Abnormality of Gait as a Predictor of Non-Alzheimer's Dementia

Thu, 28Â NovÂ 2002 00:00:00 -05:00

Background: Neurologic abnormalities affecting gait occur early in several types of non-Alzheimer's dementias, but their value in predicting the development of dementia is uncertain.

Methods: We analyzed the relation between neurologic...

PERSPECTIVE: Mercury and Health

Thu, 28Â NovÂ 2002 00:00:00 -05:00

Mercury, particularly methylmercury, is an established worldwide environmental pollutant with known toxicity in humans. The toxic effects of methylmercury in fish were first brought to light after several episodes of...

SOUNDING BOARD: Transient Ischemic Attack â Proposal for a New Definition

Thu, 21Â NovÂ 2002 00:00:00 -05:00

The definition of transient ischemic attack (TIA) and the assumptions underlying the definition have been out of date for some time and are no longer consistent with current concepts of...

CLINICAL PRACTICE: Transient Ischemic Attack

S. Claiborne Johnston, M.D., Ph.D. — Thu, 21Â NovÂ 2002 00:00:00 -05:00

A 72-year-old woman telephones her physician immediately after recovering from a 30-minute episode of difficulty speaking and weakness of the right side of the face and right arm. Her medical...

ORIGINAL ARTICLE: Normal Vision despite Narrowing of the Optic Canal in Fibrous Dysplasia

Thu, 21Â NovÂ 2002 00:00:00 -05:00

Background: Fibrous dysplasia of bone frequently involves the anterior base of the cranium and results in encasement of the optic-nerve canals. It has been assumed that such encasement leads to...

PERSPECTIVE: Transient Ischemic Attacks

Thu, 21Â NovÂ 2002 00:00:00 -05:00

The occurrence of fleeting episodes of numbness or paralysis was known to early physicians. One referred to them as âstraws which show how the intracranial wind is blowing.â In 1950,...

EDITORIAL: Corticosteroids for Everyone with Meningitis?

Thu, 14Â NovÂ 2002 00:00:00 -05:00

In 1988, a landmark study was reported on the use of adjunctive treatment with dexamethasone in infants and children who had bacterial meningitis.1 The rationale for this study was based...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 35-2002 â A Nine-Year-Old Girl with Cold Intolerance, Visual-Field Defects, and a Suprasellar Tumor

R. Michael Scott and E. Tessa Hedley-Whyte — Thu, 14Â NovÂ 2002 00:00:00 -05:00

A nine-year-old right-handed girl was admitted to the hospital because of a suprasellar mass.

The girl had been well, with normal development, until about six months earlier, when her mother observed...

ORIGINAL ARTICLE: Dexamethasone in Adults with Bacterial Meningitis

Thu, 14Â NovÂ 2002 00:00:00 -05:00

Background: Mortality and morbidity rates are high among adults with acute bacterial meningitis, especially those with pneumococcal meningitis. In studies of bacterial meningitis in animals, adjuvant treatment with corticosteroids has...

ORIGINAL ARTICLE: Two Patients with Unusual Forms of VaricellaâZoster Virus Vasculopathy

Thu, 07Â NovÂ 2002 00:00:00 -05:00

Infection of cerebral arteries by varicellaâzoster virus (VZV) can produce unifocal or multifocal vasculopathy. Unifocal large-vessel vasculopathy (granulomatous arteritis) usually affects elderly immunocompetent persons, whereas multifocal vasculopathy occurs primarily in...

ORIGINAL ARTICLE: A Population-Based Study of Measles, Mumps, and Rubella Vaccination and Autism

Thu, 07Â NovÂ 2002 00:00:00 -05:00

Background: It has been suggested that vaccination against measles, mumps, and rubella (MMR) is a cause of autism.

Methods: We conducted a retrospective cohort study of all children born in Denmark...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 34-2002 â A 55-Year-Old Man with Cognitive and Sensorimotor Findings and Intracranial Lesions

Paula Ravin and E. Tessa Hedley-Whyte — Thu, 31Â OctÂ 2002 00:00:00 -05:00

A 55-year-old right-handed man was admitted to the hospital because of difficulty with his handwriting and motor problems in his right leg.

The patient, who was a teacher, had been well...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 33-2002 â A 28-Year-Old Woman with Ocular Inflammation, Fever, and Headache

Stuart B. Mushlin, Jeffrey M. Drazen, Martin A. Samuels, and Eugene J. Mark — Thu, 24Â OctÂ 2002 00:00:00 -05:00

A 28-year-old woman was admitted to the hospital because of ocular inflammation, fever, and headache.

The patient had been well until three weeks earlier, when a cough developed, initially productive of...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 31-2002 â A 61-Year-Old Man with Headache and Multiple Infarcts

Martin A. Samuels, Mary Etta King, and Ulysses Balis — Thu, 10Â OctÂ 2002 00:00:00 -05:00

A 61-year-old man was admitted to the hospital because of right hemiplegia, aphasia, and obtundation.

According to the patient's family, he had been well at his home in Haiti until about...

EDITORIAL: Hyperbaric-Oxygen Therapy for Acute Carbon Monoxide Poisoning

Thu, 03Â OctÂ 2002 00:00:00 -05:00

Carbon monoxide appears to be the leading cause of injury and death due to poisoning worldwide.1 A colorless, odorless, tasteless gas, carbon monoxide is produced by the incomplete combustion of...

REVIEW ARTICLE: Analgesics for the Treatment of Pain in Children

Charles B. Berde, M.D., Ph.D., and Navil F. Sethna, M.B., Ch.B. — Thu, 03Â OctÂ 2002 00:00:00 -05:00

Treatment of pain and suffering should be a priority for all clinicians. Previous reviews1 decried inadequate treatment of pain in infants and children. Surveys in the 1970s and 1980s2 reported...

ORIGINAL ARTICLE: Hyperbaric Oxygen for Acute Carbon Monoxide Poisoning

Thu, 03Â OctÂ 2002 00:00:00 -05:00

Background: Patients with acute carbon monoxide poisoning commonly have cognitive sequelae. We conducted a double-blind, randomized trial to evaluate the effect of hyperbaric-oxygen treatment on such cognitive sequelae.

Methods: We randomly...

PERSPECTIVE: Carbon Monoxide Poisoning

Thu, 03Â OctÂ 2002 00:00:00 -05:00

Carbon monoxide poisoning is the most common type of accidental poisoning in the United States, accounting for thousands of emergency department visits and some 800 deaths annually. Carbon monoxide, an...

EDITORIAL: Syncope â Getting to the Heart of the Matter

Thu, 19Â SepÂ 2002 00:00:00 -05:00

Syncope is the sudden and transient loss of consciousness accompanied by a loss of postural tone. The term derives from the Greek word synkoptein, meaning âto cut short,â and purportedly,...

REVIEW ARTICLE: Tinnitus

Alan H. Lockwood, M.D., Richard J. Salvi, Ph.D., and Robert F. Burkard, Ph.D. — Thu, 19Â SepÂ 2002 00:00:00 -05:00

Tinnitus is a common yet poorly understood disorder. Data from the National Center for Health Statistics show that tinnitus is more common in men than women and increases in prevalence...

IMAGES IN CLINICAL MEDICINE: Ulnar-Nerve Schwannoma

James Chang, M.D. and Matthew B. Klein, M.D. — Thu, 19Â SepÂ 2002 00:00:00 -05:00

Figure 1. A 29-year-old right-handed woman with a five-year history of a slowly enlarging mass on the left palm had a 2-by-2-cm mass over Guyon's canal in the left wrist...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 27-2002 â A 5 1/2-Year-Old Boy with Seizures and Progressive Deterioration of Cognitive and Motor Function

Robert S. Rust and Diane Karluk — Thu, 29Â AugÂ 2002 00:00:00 -05:00

A 5 1/2-year-old boy was admitted for video electroencephalographic monitoring to investigate his seizures.

The boy had been born to a 21-year-old woman by spontaneous vaginal delivery after an uncomplicated, full-term,...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 25-2002 â A 46-Year-Old Woman with Extensive Pulmonary Infiltrates

Katherine McGowan and Eugene J. Mark — Thu, 15Â AugÂ 2002 00:00:00 -05:00

A 46-year-old woman was admitted to the hospital because of extensive pulmonary infiltrates.

The patient had felt well until several weeks before admission, when she began to have abdominal swelling and...

ORIGINAL ARTICLE: Intramuscular Injection of Botulinum Toxin for the Treatment of Wrist and Finger Spasticity after a Stroke

Thu, 08Â AugÂ 2002 00:00:00 -05:00

Background: Spasticity is a disabling complication of stroke, and it is uncertain whether intramuscular injections of botulinum toxin type A reduce disability in persons with spasticity of the wrist and...

PERSPECTIVE: Stroke, Spasticity, and Botulinum Toxin

Thu, 08Â AugÂ 2002 00:00:00 -05:00

Botulinum toxin is in the news these days. First came September 11 and fears of chemical terrorism. One gram of this âmost poisonous poisonâ could kill a million people were...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Knocking Out the DREAM to Study Pain

Thu, 01Â AugÂ 2002 00:00:00 -05:00

Although some mechanisms by which acute pain evolves into a chronic syndrome are known, many others, including changes in the brain stem, thalamus, and cerebral cortex, are not understood. Moreover,...

CLINICAL PRACTICE: Herpes Zoster

John W. Gnann, Jr., M.D., and Richard J. Whitley, M.D. — Thu, 01Â AugÂ 2002 00:00:00 -05:00

A 77-year-old man reports a five-day history of burning and aching pain in his right side and a two-day history of erythema and clusters of clear vesicles, accompanied by headache...

ORIGINAL ARTICLE: Risperidone in Children with Autism and Serious Behavioral Problems

Thu, 01Â AugÂ 2002 00:00:00 -05:00

Background: Atypical antipsychotic agents, which block postsynaptic dopamine and serotonin receptors, have advantages over traditional antipsychotic medications in the treatment of adults with schizophrenia and may be beneficial in children...

PERSPECTIVE: The Autistic-Spectrum Disorders

Thu, 01Â AugÂ 2002 00:00:00 -05:00

Any person, talented or handicapped, whose social skills have been severely deficient since very early childhood, who started to talk late or whose communicative use of language is inadequate, and...

ORIGINAL ARTICLE: Dermoid Inclusion Cysts and Early Spinal Cord Tethering after Fetal Surgery for Myelomeningocele

Thu, 25Â JulÂ 2002 00:00:00 -05:00

02072523.xml Myelomeningocele occurs in approximately 1 of every 2000 live births.1 The prenatal incidence is higher, because many parents elect to terminate the pregnancy, once the diagnosis has been...

REVIEW ARTICLE: Heat Stroke

Abderrezak Bouchama, M.D., and James P. Knochel, M.D. — Thu, 20Â JunÂ 2002 00:00:00 -05:00

Heat stroke is a life-threatening illness characterized by an elevated core body temperature that rises above 40Â°C and central nervous system dysfunction that results in delirium, convulsions, or coma.1 02062024.xml...

EDITORIAL: Sporadic Cases of Hereditary Systemic Amyloidosis

Thu, 06Â JunÂ 2002 00:00:00 -05:00

The systemic amyloidoses comprise a spectrum of acquired and hereditary diseases caused by the deposition of characteristic fibrillar material in various organs and tissues throughout the body. These fibrils are...

CLINICAL PRACTICE: Carpal Tunnel Syndrome

Jeffrey N. Katz, M.D., and Barry P. Simmons, M.D. — Thu, 06Â JunÂ 2002 00:00:00 -05:00

A 64-year-old, right-handed, retired woman presents with intermittent numbness, tingling, and burning pain in the three radial digits of both hands. She has had these symptoms for three months, and...

ORIGINAL ARTICLE: Central Nervous System Infection in Congenital Syphilis

Thu, 06Â JunÂ 2002 00:00:00 -05:00

Background: Identification of infants with Treponema pallidum infection of the central nervous system remains challenging.

Methods: We used rabbit-infectivity testing of the cerebrospinal fluid to detect T. pallidum infection of the...

ORIGINAL ARTICLE: Misdiagnosis of Hereditary Amyloidosis as AL (Primary) Amyloidosis

Thu, 06Â JunÂ 2002 00:00:00 -05:00

Background: Hereditary, autosomal dominant amyloidosis, caused by mutations in the genes encoding transthyretin, fibrinogen A Î±-chain, lysozyme, or apolipoprotein A-I, is thought to be extremely rare and is not routinely...

EDITORIAL: Physician-Assisted Death â A Last Resort?

Thu, 23Â MayÂ 2002 00:00:00 -05:00

During the past decade, patients with the uncommon disease amyotrophic lateral sclerosis (ALS) have had a prominent role in the debate about physician-assisted suicide and voluntary active euthanasia. In Canada...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 16-2002 â A 41-Year-Old Woman with Global Headache and an Intracranial Mass

David E. Thaler and Matthew P. Frosch — Thu, 23Â MayÂ 2002 00:00:00 -05:00

A 41-year-old woman was admitted to the hospital because of an intracranial mass.

The patient had been in excellent health until one week earlier, when a global headache developed, accompanied by...

SPECIAL ARTICLE: Euthanasia and Physician-Assisted Suicide among Patients with Amyotrophic Lateral Sclerosis in the Netherlands

Thu, 23Â MayÂ 2002 00:00:00 -05:00

Background: Amyotrophic lateral sclerosis (ALS) is a disease that causes progressive paralysis leading to respiratory failure. Patients with ALS may consider physician-assisted suicide. However, it is not known how many...

IMAGES IN CLINICAL MEDICINE: Neonatal Vein of Galen Malformation

Bernhard Fischer, M.D. and Stefan Palkovic, M.D., Ph.D. — Thu, 16Â MayÂ 2002 00:00:00 -05:00

Figure 1. Sonographic imaging of the brain of a neonate showed a dilated hypoechoic venous lesion within the tentorial hiatus. Computed tomographic (CT) angiography demonstrated a large arteriovenous malformation of...

IMAGES IN CLINICAL MEDICINE: Herpes Zoster Ophthalmicus Followed by Contralateral Hemiparesis

Raul G. Nogueira, M.D. and Volney L. Sheen, M.D., Ph.D. — Thu, 11Â AprÂ 2002 00:00:00 -05:00

Figure 1. A 71-year-old man with a history of multiple myeloma and subsequent autologous bone marrow transplantation was evaluated for a facial rash in the distribution of the ophthalmic branch...

ORIGINAL ARTICLE: Neuroblastoma Screening at One Year of Age

Thu, 04Â AprÂ 2002 00:00:00 -05:00

Background: Neuroblastoma is the second most common type of childhood tumor. It is not known whether screening for neuroblastoma at one year of age reduces the incidence of metastatic disease...

ORIGINAL ARTICLE: Screening of Infants and Mortality Due to Neuroblastoma

Thu, 04Â AprÂ 2002 00:00:00 -05:00

Background: Neuroblastoma, the most common extracranial solid tumor that occurs in early childhood, can be identified in the preclinical stages by the detection of catecholamines in the urine. However, it...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 10-2002 â A 52-Year-Old Woman with Recurrent Unsteadiness, Slurred Speech, and Fatigue

Lloyd M. Alderson and Ivana Delalle — Thu, 28Â MarÂ 2002 00:00:00 -05:00

A 52-year-old right-handed woman was admitted to the neurologic clinic because of ataxia and fatigue.

The patient had been well until four months earlier, when she had had a Ã¢â¬Åsinus infectionÃ¢â¬Â...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 9-2002 â An 80-Year-Old Woman with Sudden Unilateral Blindness

Don C. Bienfang and Diane Karluk — Thu, 21Â MarÂ 2002 00:00:00 -05:00

An 80-year-old woman was admitted to the hospital because of sudden unilateral blindness.

The patient had a long history of medical problems. These included hypertension; mitral and tricuspid regurgitation, which had...

ORIGINAL ARTICLE: An Outbreak of Eosinophilic Meningitis Caused by in Travelers Returning from the Caribbean

Thu, 28Â FebÂ 2002 00:00:00 -05:00

Background: Outbreaks of eosinophilic meningitis caused by the roundworm Angiostrongylus cantonensis are rarely reported, even in regions of endemic infection such as Southeast Asia and the Pacific Basin. We report...

EDITORIAL: Therapeutic Hypothermia after Cardiac Arrest

Thu, 21Â FebÂ 2002 00:00:00 -05:00

In this issue of the Journal, the reported results of two randomized clinical trials, one in Australia1 and the other in Europe,2 showed a neurologic benefit of mild therapeutic hypothermia...

ORIGINAL ARTICLE: Treatment of Comatose Survivors of Out-of-Hospital Cardiac Arrest with Induced Hypothermia

Thu, 21Â FebÂ 2002 00:00:00 -05:00

Background: Cardiac arrest outside the hospital is common and has a poor outcome. Studies in laboratory animals suggest that hypothermia induced shortly after the restoration of spontaneous circulation may improve...

ORIGINAL ARTICLE: Mild Therapeutic Hypothermia to Improve the Neurologic Outcome after Cardiac Arrest

The Hypothermia after Cardiac Arrest Study Group — Thu, 21Â FebÂ 2002 00:00:00 -05:00

Background: Cardiac arrest with widespread cerebral ischemia frequently leads to severe neurologic impairment. We studied whether mild systemic hypothermia increases the rate of neurologic recovery after resuscitation from cardiac arrest...

PERSPECTIVE: Hypothermia to Protect the Brain

Thu, 21Â FebÂ 2002 00:00:00 -05:00

For decades, cooling the body below the normal physiologic temperature has been used as a therapeutic tool. Hypothermia is used most often during cardiac surgery with cardiopulmonary bypass, as a...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 5-2002 â A 15-Year-Old Boy with a Retro-orbital Mass and Impaired Vision

Craig A. Hurwitz, M.D.,and William C. Faquin, M.D., Ph.D. — Thu, 14Â FebÂ 2002 00:00:00 -05:00

A 15-year-old boy was admitted to the hospital because of a retro-orbital mass.

The patient had been well until three weeks earlier, when he began to have right retro-orbital and frontal...

IMAGES IN CLINICAL MEDICINE: An Unusual Saccular Aneurysm

Peter Vajkoczy, M.D. and Marius Hartmann, M.D. — Thu, 14Â FebÂ 2002 00:00:00 -05:00

Figure 1. A 49-year-old woman underwent a diagnostic workup for recurrent headaches. Computed tomography suggested the presence of an incidental aneurysm of the left internal carotid artery. This finding was...

ORIGINAL ARTICLE: Plasma Homocysteine as a Risk Factor for Dementia and Alzheimer's Disease

Thu, 14Â FebÂ 2002 00:00:00 -05:00

Background: 02021427.xml In cross-sectional studies, elevated plasma homocysteine levels have been associated with poor cognition and dementia. Studies of newly diagnosed dementia are required in order to establish whether the...

PERSPECTIVE: Homocysteine and Dementias

Thu, 14Â FebÂ 2002 00:00:00 -05:00

Homocysteine is a simple amino acid that has recently received a great deal of attention as a risk factor for atherothrombotic vascular disease. There is reasonably compelling epidemiologic evidence of...

ORIGINAL ARTICLE: Expression of p53 and Prognosis in Children with Malignant Gliomas

Thu, 07Â FebÂ 2002 00:00:00 -05:00

Background: The prognosis of children with high-grade gliomas is uncertain, even when clinical and histologic findings are considered. We investigated whether mutations in the TP53 gene or the degree of...

REVIEW ARTICLE: Migraine â Current Understanding and Treatment

Thu, 24Â JanÂ 2002 00:00:00 -05:00

Migraine is a common, chronic, incapacitating neurovascular disorder, characterized by attacks of severe headache, autonomic nervous system dysfunction, and in some patients, an aura involving neurologic symptoms.12 Recent advances in...

ORIGINAL ARTICLE: A Deletion Involving the Connexin 30 Gene in Nonsyndromic Hearing Impairment

Thu, 24Â JanÂ 2002 00:00:00 -05:00

Background: Inherited hearing impairment affects about 1 in 2000 newborns. Up to 50 percent of all patients with autosomal recessive nonsyndromic prelingual deafness in different populations have mutations in the...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 2-2002 â A 58-Year-Old Woman with Unilateral Loss of Vision and Postmenopausal Bleeding

Donald J. D'Amico, M.D., and Thaddeus Dryja, M.D. — Thu, 17Â JanÂ 2002 00:00:00 -05:00

A 58-year-old woman was evaluated at the hospital because of loss of vision in her right eye.

The patient had been in good general health until six months earlier, when narrow-angle...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 1-2002 â A 24-Year-Old Woman with Paresthesias and Muscle Cramps after a Stay in Africa

Thomas B. Nutman, M.D., and Richard L. Kradin, M.D. — Thu, 10Â JanÂ 2002 00:00:00 -05:00

A 24-year-old woman was admitted to the Travel and Tropical Medicine Center at this hospital because of edema and intermittent paresthesias of the right leg.

The patient was a volunteer with...

IMAGES IN CLINICAL MEDICINE: Syringomyelia

Jose Alberto SagÃ¡stegui-RodrÃguez, M.D. and Salvador Cruz-Flores, M.D. — Thu, 03Â JanÂ 2002 00:00:00 -05:00

Figure 1. A 24-year-old man had a three-year history of progressive muscle wasting and sensory loss in his arms, dysphonia, and dysphagia. During the previous five months, he had had...

ORIGINAL ARTICLE: High-Dose Intravenous Immune Globulin for Stiff-Person Syndrome

Thu, 27Â DecÂ 2001 00:00:00 -05:00

Background: Stiff-person syndrome is a disabling central nervous system disorder with no satisfactory treatment that is characterized by muscle rigidity, episodic muscle spasms, high titers of antibodies against glutamic acid...

EDITORIAL: Computed Tomography of the Head before a Lumbar Puncture in Suspected Meningitis â Is It Helpful?

Thu, 13Â DecÂ 2001 00:00:00 -05:00

Meningitis remains a formidable foe because it often progresses rapidly and causes substantial morbidity and mortality.123 The rational management of meningitis has generally included a lumbar puncture to substantiate the...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 38-2001 â A 51-Year-Old Woman with Lung Cancer and Neuropsychiatric Abnormalities

Anthony A. Amato, M.D., and Matthew P. Anderson, M.D., Ph.D. — Thu, 13Â DecÂ 2001 00:00:00 -05:00

A 51-year-old woman was admitted to the hospital because of paresthesias, ataxic gait, and hallucinations.

The patient had been well until 9 or 10 months earlier, when tingling and numbness developed...

ORIGINAL ARTICLE: Computed Tomography of the Head before Lumbar Puncture in Adults with Suspected Meningitis

Thu, 13Â DecÂ 2001 00:00:00 -05:00

Background: In adults with suspected meningitis clinicians routinely order computed tomography (CT) of the head before performing a lumbar puncture.

Methods: We prospectively studied 301 adults with suspected meningitis to determine...

EDITORIAL: Do Nonsteroidal Antiinflammatory Drugs Reduce the Risk of Alzheimer's Disease?

Thu, 22Â NovÂ 2001 00:00:00 -05:00

In this issue of the Journal, in 't Veld and colleagues1 report the results of a long-term prospective study of the relation between the use of nonsteroidal antiinflammatory drugs (NSAIDs)...

ORIGINAL ARTICLE: Nonsteroidal Antiinflammatory Drugs and the Risk of Alzheimer's Disease

Thu, 22Â NovÂ 2001 00:00:00 -05:00

Background: Previous studies have suggested that the use of nonsteroidal antiinflammatory drugs (NSAIDs) may help to prevent Alzheimer's disease. The results, however, have been inconsistent.

Methods: We studied the association between...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 35-2001 â An 81-Year-Old Man with Hypothermia, Bradycardia, and Confusion

Steven K. Grinspoon and E. Tessa Hedley-White — Thu, 15Â NovÂ 2001 00:00:00 -05:00

An 81-year-old man was transferred to the hospital because of bradycardia, hypothermia, and increasing confusion.

A platelet count of 85,000 per cubic millimeter had been discovered five years before admission, but...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 33-2001 â A 33-Year-Old Man with a Rash, Pulmonary Infection, and Neurologic Disorder

C. Fordham von Reyn, M.D., and Eugene J. Mark, M.D. — Thu, 25Â OctÂ 2001 00:00:00 -05:00

A 33-year-old man was admitted to the hospital because of leg numbness and back pain.

The patient had been well until six weeks before admission, when pruritus developed on his upper...

REVIEW ARTICLE: Tourette's Syndrome

Joseph Jankovic, M.D. — Thu, 18Â OctÂ 2001 00:00:00 -05:00

Tourette's syndrome is a neurologic disorder named after the French neurologist Georges Gilles de la Tourette, who, in 1885, described nine patients with childhood-onset tics, accompanied in some by uncontrollable...

IMAGES IN CLINICAL MEDICINE: Hemorrhagic Transformation of a Cerebral Infarct

Scott Silliman, M.D. and Michael Blum, M.D. — Thu, 11Â OctÂ 2001 00:00:00 -05:00

Figure 1. A 74-year-old woman presented to the emergency department two hours after the development of slurred speech and weakness of the left arm and leg. The blood pressure was...

CLINICAL PRACTICE: Essential Tremor

Elan D. Louis, M.D. — Thu, 20Â SepÂ 2001 00:00:00 -05:00

A 66-year-old woman presents with tremor of the hands. She first noticed a mild tremor six years earlier, but the tremor has been worsening for the past two years. It...

ORIGINAL ARTICLE: Medical Treatment for Neurocysticercosis Characterized by Giant Subarachnoid Cysts

Thu, 20Â SepÂ 2001 00:00:00 -05:00

Background: Infection with the larval form of the pork tapeworm, Taenia solium, can lead to the development of cysts in the brain. Surgical removal of cysts has been the accepted...

REVIEW ARTICLE: Toxic Leukoencephalopathy

Christopher M. Filley, M.D., and B.K. Kleinschmidt-DeMasters, M.D. — Thu, 09Â AugÂ 2001 00:00:00 -05:00

Leukoencephalopathy is a structural alteration of cerebral white matter in which myelin suffers the most damage. Toxic leukoencephalopathy may be caused by exposure to a wide variety of agents, including...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 21-2001 â A 31-Year-Old Man with an Apparent Seizure and a Mass in the Right Parietal Lobe

Allan H. Ropper, M.D., and Anat Stemmer-Rachamimov, M.D. — Thu, 12Â JulÂ 2001 00:00:00 -05:00

A 31-year-old man was admitted to the hospital because of an apparent seizure and radiologic evidence of a cerebral lesion.

The patient had been in excellent health until five days earlier,...

EDITORIAL: Hemiplegic Migraine â Downstream of a Single-Base Change

Thu, 05Â JulÂ 2001 00:00:00 -05:00

Hemiplegic migraine (with or without cerebellar signs), spinocerebellar ataxia, and episodic ataxia can all result from changes in the gene that encodes the P/Q-type neuronal calcium channel, just one of...

IMAGES IN CLINICAL MEDICINE: Vomiting of Neurologic Origin

Juan C. GarcÃa-MoncÃ³, M.D. and Jose A. Larena, M.D. — Thu, 05Â JulÂ 2001 00:00:00 -05:00

Figure 1. A 25-year-old man had a seven-month history of repeated, unprovoked, sudden vomiting without accompanying headache or any other neurologic or gastrointestinal symptoms. Vomiting had occurred daily in the...

ORIGINAL ARTICLE: The Clinical Spectrum of Familial Hemiplegic Migraine Associated with Mutations in a Neuronal Calcium Channel

Thu, 05Â JulÂ 2001 00:00:00 -05:00

Background: Familial hemiplegic migraine, an autosomal dominant disorder characterized by attacks of transient hemiparesis followed by a migraine headache, is divided into pure familial hemiplegic migraine (affecting 80 percent of...

REVIEW ARTICLE: Amyotrophic Lateral Sclerosis

Lewis P. Rowland, M.D., and Neil A. Shneider, M.D., Ph.D. — Thu, 31Â MayÂ 2001 00:00:00 -05:00

Charcot described amyotrophic lateral sclerosis (ALS) in 1874. Despite progress, this creeping paralysis, known colloquially as Lou Gehrig's disease, is still not visibly affected by available therapies. However, advances in...

EDITORIAL: Controlling New Prion Diseases

Thu, 17Â MayÂ 2001 00:00:00 -05:00

Like the murrain â the fifth plague in the biblical story of Exodus, which targeted cattle â a new prion disease called bovine spongiform encephalopathy (BSE) has had a devastating...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 15-2001 â A 72-Year-Old Man with Persistent Fever and Hypotension

Ursula B. Kaiser, E. Tessa Hedley-Whyte — Thu, 17Â MayÂ 2001 00:00:00 -05:00

A 72-year-old man was admitted to the hospital because of persistent fever and hypotension.

The patient had been well until eight days earlier, when nausea and vomiting developed, with lower abdominal...

SPECIAL ARTICLE: Neurodegenerative Diseases and Prions

Stanley B. Prusiner, M.D. — Thu, 17Â MayÂ 2001 00:00:00 -05:00

Twenty-five years ago, little was known about the causes of neurodegenerative diseases. Now, however, it is clear that they result from abnormalities in the processing of proteins. In each of...

REVIEW ARTICLE: Spontaneous Intracerebral Hemorrhage

Thu, 10Â MayÂ 2001 00:00:00 -05:00

Nontraumatic intracerebral hemorrhage is bleeding into the parenchyma of the brain that may extend into the ventricles and, in rare cases, the subarachnoid space. Each year, approximately 37,000 to 52,400...

IMAGES IN CLINICAL MEDICINE: Papilledema Associated with Intracranial Hypertension

William J. Power, F.R.C.S. and Edward Harris, M.D. — Thu, 10Â MayÂ 2001 00:00:00 -05:00

Figure 1. A 21-year-old woman presented with a two-month history of gradual loss of vision in both eyes and increasing headaches. A ventriculoperitoneal shunt had been inserted when she was...

EDITORIAL: Brain Death â Well Settled yet Still Unresolved

Thu, 19Â AprÂ 2001 00:00:00 -05:00

If one subject in health law and bioethics can be said to be at once well settled and persistently unresolved, it is how to determine that death has occurred. Once...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 12-2001 â A 16-Year-Old Boy with an Altered Mental Status and Muscle Rigidity

Mark H. Libenson, Jane M. Yang — Thu, 19Â AprÂ 2001 00:00:00 -05:00

A 16-year-old boy was admitted to the hospital because of an altered mental and emotional status.

At an uncertain interval before admission, the patient began to have diarrhea. One week before...

REVIEW ARTICLE: The Diagnosis of Brain Death

Eelco F.M. Wijdicks, M.D. — Thu, 19Â AprÂ 2001 00:00:00 -05:00

Physicians, health care workers, members of the clergy, and laypeople throughout the world have accepted fully that a person is dead when his or her brain is dead. In the...

ORIGINAL ARTICLE: The Teratogenicity of Anticonvulsant Drugs

Thu, 12Â AprÂ 2001 00:00:00 -05:00

Background: The frequency of major malformations, growth retardation, and hypoplasia of the midface and fingers, known as anticonvulsant embryopathy, is increased in infants exposed to anticonvulsant drugs in utero. However,...

ORIGINAL ARTICLE: A Reevaluation of the Duration of Survival after the Onset of Dementia

Thu, 12Â AprÂ 2001 00:00:00 -05:00

Background: Dementia shortens life expectancy; estimates of median survival after the onset of dementia have ranged from 5 to 9.3 years. Previous studies of people with existing dementia, however, may...

CLINICAL PROBLEM-SOLVING: Less is More

C. Christopher Smith, M.D., Jess Mandel, M.D., and Booker Bush, M.D. — Thu, 05Â AprÂ 2001 00:00:00 -05:00

A 63-year-old woman presented to her physician with fatigue, weakness, anorexia, and an unintentional weight loss of 14.5 kg (32 lb) during the previous four months. She reported increased constipation...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 9-2001 â A 64-Year-Old Woman with Peripheral Neuropathy, Paraproteinemia, and Lymphadenopathy

Kenneth C. Gorson, E. Tessa Hedley-Whyte, Martha M. Skinner — Thu, 22Â MarÂ 2001 00:00:00 -05:00

A 64-year-old, right-handed woman was admitted to the hospital because of motor and sensory impairment in her right arm.

Two years before admission, generalized lymphadenopathy had been diagnosed. At that time,...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 8-2001 â A 61-Year-Old Man with Transient Quadriplegia and Apnea

Eric T.Wong, David N. Louis — Thu, 15Â MarÂ 2001 00:00:00 -05:00

A 61-year-old man was admitted to the hospital because of transient quadriplegia and apnea.

The patient had been well until the morning of admission, when stiffness and pain developed in his...

IMAGES IN CLINICAL MEDICINE: Four Types of Acute Post-Traumatic Intracranial Hemorrhage

Julian A. Mattiello, M.D. and Michael Munz, M.D. — Thu, 22Â FebÂ 2001 00:00:00 -05:00

Figure 1. A 71-year-old man was pushed onto his back by a slow-moving vehicle. Initially, he was alert and fully oriented. In the emergency department, the patient was disoriented to...

EDITORIAL: The Apgar Score in the 21st Century

Thu, 15Â FebÂ 2001 00:00:00 -05:00

In 1952, Dr. Virginia Apgar developed a scoring system to evaluate the condition of neonates born at the Sloane Hospital for Women in New York City.1 Dr. Apgar, an obstetrical...

ORIGINAL ARTICLE: The Continuing Value of the Apgar Score for the Assessment of Newborn Infants

Brian M. Casey, M.D., Donald D. McIntire, Ph.D., and Kenneth J. Leveno, M.D. — Thu, 15Â FebÂ 2001 00:00:00 -05:00

Background: The 10-point Apgar score has been used to assess the condition and prognosis of newborn infants throughout the world for almost 50 years. Some investigators have proposed that measurement...

EDITORIAL: Coronary-Artery Bypass Surgery and the Brain

Thu, 08Â FebÂ 2001 00:00:00 -05:00

Coronary-artery bypass grafting (CABG) is a very effective procedure for reducing angina and stabilizing ventricular function. It is not surprising, therefore, that more than 500,000 bypass procedures are performed in...

ORIGINAL ARTICLE: Longitudinal Assessment of Neurocognitive Function after Coronary-Artery Bypass Surgery

Thu, 08Â FebÂ 2001 00:00:00 -05:00

Background: Cognitive decline complicates early recovery after coronary-artery bypass grafting (CABG) and may be evident in as many as three quarters of patients at the time of discharge from the...

EDITORIAL: Predicting Cerebral Edema during Diabetic Ketoacidosis

Thu, 25Â JanÂ 2001 00:00:00 -05:00

Cerebral edema is a devastating complication of diabetic ketoacidosis and remains the leading cause of serious illness and death in children with diabetes mellitus.1 The causes of cerebral edema are...

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 3-2001 â A 59-Year-Old Diabetic Man with Unilateral Visual Loss and Oculomotor-Nerve Palsy

Richard B. Brown, Stephen K. Lau — Thu, 25Â JanÂ 2001 00:00:00 -05:00

A 59-year-old man was admitted to the hospital because of right-sided visual loss and oculomotor-nerve palsy.

The patient had had type 2 diabetes mellitus for 25 years; during most of those...

ORIGINAL ARTICLE: Risk Factors for Cerebral Edema in Children with Diabetic Ketoacidosis

Thu, 25Â JanÂ 2001 00:00:00 -05:00

Background: Cerebral edema is an uncommon but devastating complication of diabetic ketoacidosis in children. Risk factors for this complication have not been clearly defined.

Methods: In this multicenter study, we identified...

REVIEW ARTICLE: Syncope

Wishwa N. Kapoor, M.D., M.P.H. — Thu, 21Â DecÂ 2000 00:00:00 -05:00

Syncope is a sudden and brief loss of consciousness associated with a loss of postural tone, from which recovery is spontaneous. The pathophysiology of all forms of syncope consists of...

ORIGINAL ARTICLE: Adverse Cardiovascular and Central Nervous System Events Associated with Dietary Supplements Containing Ephedra Alkaloids

Christine A. Haller, M.D., and Neal L. Benowitz, M.D. — Thu, 21Â DecÂ 2000 00:00:00 -05:00

Background: Dietary supplements that contain ephedra alkaloids (sometimes called ma huang) are widely promoted and used in the United States as a means of losing weight and increasing energy. In...

IMAGES IN CLINICAL MEDICINE: Astrocytoma Following the Pyramidal Tract

Andreas Spuler, M.D. and John L.D. Atkinson, M.D. — Thu, 07Â DecÂ 2000 00:00:00 -05:00

Figure 1. A 57-year-old man presented with a moderate left-sided hemiparesis. T1-weighted magnetic resonance images with gadolinium revealed a contrast-enhanced lesion in the internal capsule and the adjacent basal ganglia....

ORIGINAL ARTICLE: Folic Acid Antagonists during Pregnancy and the Risk of Birth Defects

Thu, 30Â NovÂ 2000 00:00:00 -05:00

Background: Multivitamin supplementation in pregnant women may reduce the risks of cardiovascular defects, oral clefts, and urinary tract defects in their infants. We evaluated whether the folic acid component of...

EDITORIAL: A New Treatment for Postherpetic Neuralgia

Thu, 23Â NovÂ 2000 00:00:00 -05:00

Postherpetic neuralgia is the most frequent and feared complication of herpes zoster, which is the most common neurologic illness in the United States.1 Data from the VZV (Varicella Zoster Virus)...

ORIGINAL ARTICLE: Intrathecal Methylprednisolone for Intractable Postherpetic Neuralgia

Thu, 23Â NovÂ 2000 00:00:00 -05:00

Background: There is no effective treatment for intractable postherpetic neuralgia. Because there is evidence that postherpetic neuralgia has an inflammatory component, we assessed treatment with intrathecally administered methylprednisolone to reduce...

IMAGES IN CLINICAL MEDICINE: Bartonella Neuroretinitis

Kenneth C. Earhart, M.D. and Michael H. Power, M.D. — Thu, 16Â NovÂ 2000 00:00:00 -05:00

Figure 1. A 26-year-old man who presented with blurred vision was found to have bilateral papilledema and diminished visual acuity (20/60). Magnetic resonance imaging of the brain and orbits was...

ORIGINAL ARTICLE: Inactivation of the DNA-Repair Gene and the Clinical Response of Gliomas to Alkylating Agents

Thu, 09Â NovÂ 2000 00:00:00 -05:00

Background: The DNA-repair enzyme O 6-methylguanine-DNA methyltransferase (MGMT) inhibits the killing of tumor cells by alkylating agents. MGMT activity is controlled by a promoter; methylation of the promoter silences the...

ORIGINAL ARTICLE: Effects of Intrathecal Morphine on the Ventilatory Response to Hypoxia

Thu, 26Â OctÂ 2000 00:00:00 -05:00

Background: Intrathecal administration of morphine produces intense analgesia, but it depresses respiration, an effect that can be life-threatening. Whether intrathecal morphine affects the ventilatory response to hypoxia, however, is not...

IMAGES IN CLINICAL MEDICINE: A Medical Mystery

Gerhard Kurlemann, M.D. and Otfried Debus, M.D. — Thu, 05Â OctÂ 2000 00:00:00 -05:00

Figure 1. What do these photographs of the eye of a 19-year-old man show, and what systemic disorder does the man have?...

ORIGINAL ARTICLE: The Neuropathic Postural Tachycardia Syndrome

Thu, 05Â OctÂ 2000 00:00:00 -05:00

Background: The postural tachycardia syndrome is a common disorder that is characterized by chronic orthostatic symptoms and a dramatic increase in heart rate on standing, but that does not involve...

ORIGINAL ARTICLE: Autoantibodies to Ganglionic Acetylcholine Receptors in Autoimmune Autonomic Neuropathies

Thu, 21Â SepÂ 2000 00:00:00 -05:00

Background: Idiopathic autonomic neuropathy is a severe, subacute disorder with a presumed autoimmune basis. It is indistinguishable from the subacute autonomic neuropathy that may accompany lung cancer or other tumors....

EDITORIAL: New Treatments for Reflex Sympathetic Dystrophy

Thu, 31Â AugÂ 2000 00:00:00 -05:00

Reflex sympathetic dystrophy was first described by Mitchell, in 1864.1 It has been difficult for clinicians to diagnose this disorder because it has many variations, often follows minor injury, and...

ORIGINAL ARTICLE: Intrathecal Baclofen for the Treatment of Dystonia in Patients with Reflex Sympathetic Dystrophy

Thu, 31Â AugÂ 2000 00:00:00 -05:00

Background: Patients with reflex sympathetic dystrophy (also known as the complex regional pain syndrome) may have dystonia, which is often unresponsive to treatment. Some forms of dystonia respond to the...

ORIGINAL ARTICLE: Spinal Cord Stimulation in Patients with Chronic Reflex Sympathetic Dystrophy

Thu, 31Â AugÂ 2000 00:00:00 -05:00

Background: Chronic reflex sympathetic dystrophy (also called the complex regional pain syndrome) is a painful, disabling disorder for which there is no proven treatment. In observational studies, spinal cord stimulation...

EDITORIAL: Detection of Preclinical Alzheimer's Disease

Thu, 17Â AugÂ 2000 00:00:00 -05:00

Alzheimer's disease is a chronic disorder with a gradual onset and slowly progressive course that is characterized by an inevitable deterioration in cognitive function. To develop new methods to prevent...

ORIGINAL ARTICLE: Patterns of Brain Activation in People at Risk for Alzheimer's Disease

Thu, 17Â AugÂ 2000 00:00:00 -05:00

Background: The Îµ4 allele of the apolipoprotein E gene (APOE) is the chief known genetic risk factor for Alzheimer's disease, the most common cause of dementia late in life. To...

EDITORIAL: Extremely Preterm Birth â Defining the Limits of Hope

Thu, 10Â AugÂ 2000 00:00:00 -05:00

Despite an increase in the frequency of premature birth,2 new techniques, including surfactant-replacement therapy and increased use of antenatal glucocorticoids, have led to continued improvement in the survival of extremely...

ORIGINAL ARTICLE: Neurologic and Developmental Disability after Extremely Preterm Birth

Thu, 10Â AugÂ 2000 00:00:00 -05:00

Background: Small studies show that many children born as extremely preterm infants have neurologic and developmental disabilities. We evaluated all children who were born at 25 or fewer completed weeks...

ORIGINAL ARTICLE: Validity of a Set of Clinical Criteria to Rule Out Injury to the Cervical Spine in Patients with Blunt Trauma

Thu, 13Â JulÂ 2000 00:00:00 -05:00

Background: Because clinicians fear missing occult cervical-spine injuries, they obtain cervical radiographs for nearly all patients who present with blunt trauma. Previous research suggests that a set of clinical criteria...

EDITORIAL: Who is at Low Risk after Head or Neck Trauma?

Thu, 13Â JulÂ 2000 00:00:00 -05:00

Emergency physicians have traditionally relied heavily on diagnostic imaging in the evaluation of patients with head or neck trauma. This conservative approach is intended to reduce the risk of missing...

ORIGINAL ARTICLE: Indications for Computed Tomography in Patients with Minor Head Injury

Thu, 13Â JulÂ 2000 00:00:00 -05:00

Background: Computed tomography (CT) is widely used as a screening test in patients with minor head injury, although the results are often normal. We performed a study to develop and...

REVIEW ARTICLE: Erectile Dysfunction

Tom F. Lue, M.D. — Thu, 15Â JunÂ 2000 00:00:00 -05:00

Erectile dysfunction is defined as the inability to achieve and maintain an erection sufficient to permit satisfactory sexual intercourse.1 It has been estimated to affect 20 million to 30 million...

ORIGINAL ARTICLE: Clinical Features of Nipah Virus Encephalitis among Pig Farmers in Malaysia

Thu, 27Â AprÂ 2000 00:00:00 -05:00

Background: Between September 1998 and June 1999, there was an outbreak of severe viral encephalitis due to Nipah virus, a newly discovered paramyxovirus, in Malaysia.

Methods: We studied the clinical features...

IMAGES IN CLINICAL MEDICINE: Meningeal Carcinomatosis in Breast Cancer

Deborah L. Ornstein, M.D. and Kirt Frederickson, M.D. — Thu, 13Â AprÂ 2000 00:00:00 -05:00

Figure 1. A 49-year-old woman with progressive metastatic breast cancer despite aggressive chemotherapy was hospitalized with slurred speech, clumsiness of the right hand, and increasing lethargy. Examination revealed that the...

ORIGINAL ARTICLE: Desmin Myopathy, a Skeletal Myopathy with Cardiomyopathy Caused by Mutations in the Desmin Gene

Thu, 16Â MarÂ 2000 00:00:00 -05:00

Background: Myofibrillar myopathies are a heterogeneous group of inherited or sporadic skeletal myopathies associated with cardiomyopathy. Among the myofibrillar proteins that accumulate within the muscle fibers of affected patients, the...

REVIEW ARTICLE: Neurologic Complications of the Reactivation of VaricellaâZoster Virus

Thu, 02Â MarÂ 2000 00:00:00 -05:00

Varicellaâzoster virus is an exclusively human herpesvirus that causes chickenpox (varicella), becomes latent in cranial-nerve and dorsal-root ganglia, and frequently reactivates decades later to produce shingles (zoster) and postherpetic neuralgia....

IMAGES IN CLINICAL MEDICINE: Pituitary Apoplexy

Latha Dulipsingh, M.D. and M. Nathan Lassman, M.D. — Thu, 24Â FebÂ 2000 00:00:00 -05:00

Figure 1. A 70-year-old man presented with a five-day history of worsening headaches associated with vomiting and confusion on the day of admission. He was being treated with warfarin for...

ORIGINAL ARTICLE: Orthostatic Intolerance and Tachycardia Associated with Norepinephrine-Transporter Deficiency

Thu, 24Â FebÂ 2000 00:00:00 -05:00

Background: Orthostatic intolerance is a syndrome characterized by lightheadedness, fatigue, altered mentation, and syncope and associated with postural tachycardia and plasma norepinephrine concentrations that are disproportionately high in relation to...

SOUNDING BOARD: Pharmacologic Paralysis and Withdrawal of Mechanical Ventilation at the End of Life

Thu, 17Â FebÂ 2000 00:00:00 -05:00

The right of a patient or surrogate to refuse life-sustaining treatment, including mechanical ventilation, is firmly established in American law and bioethics.1 Moreover, practice standards now encourage clinicians to administer...

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Pigment-EpitheliumâDerived Factor â A Key Coordinator of Retinal Neuronal and Vascular Functions

Thu, 03Â FebÂ 2000 00:00:00 -05:00

Precise regulation of vascular development is critical in the eye, where a few microns' deviation can cause blood vessels to move into normally avascular areas such as the cornea, vitreous,...

EDITORIAL: Lobar Intracerebral Hemorrhage

Thu, 27Â JanÂ 2000 00:00:00 -05:00

Although intracerebral hemorrhage is a less frequent cause of stroke than cerebral infarction, it is more often fatal. It is usually attributed to hypertensive small-vessel disease, and the most common...

ORIGINAL ARTICLE: Apolipoprotein E Genotype and the Risk of Recurrent Lobar Intracerebral Hemorrhage

Thu, 27Â JanÂ 2000 00:00:00 -05:00

Background: Recurrent lobar intracerebral hemorrhage is the hallmark of cerebral amyloid angiopathy. The factors that predispose patients to early recurrence of lobar hemorrhage are unknown. One candidate is the apolipoprotein...

SOUNDING BOARD: Rethinking the Role of Tube Feeding in Patients with Advanced Dementia

Thu, 20Â JanÂ 2000 00:00:00 -05:00

A byproduct of the aging of the population has been a dramatic rise in the rate of Alzheimer's disease and other types of dementia. A conservative estimate is that there...

ORIGINAL ARTICLE: A Six-Year-Old Girl with Tick Paralysis

Michael W. Felz, M.D., Carrie Davis Smith, M.D., and Thomas R. Swift, M.D. — Thu, 13Â JanÂ 2000 00:00:00 -05:00

Tick paralysis is a neurologic syndrome that is frequently confused with other acute disorders. In this syndrome, ascending paralysis is caused by a potent neurotoxin produced by an attached, engorged...

EDITORIAL: The Weak Child â A Cautionary Tale

Thu, 13Â JanÂ 2000 00:00:00 -05:00

In this issue of the Journal, Felz and colleagues describe a six-year-old child with reversible, but potentially fatal, tick paralysis that was initially thought to be the GuillainâBarrÃ© syndrome.1 A...

REVIEW ARTICLE: Avoiding Pitfalls in the Diagnosis of Subarachnoid Hemorrhage

Jonathan A. Edlow, M.D., and Louis R. Caplan, M.D. — Thu, 06Â JanÂ 2000 00:00:00 -05:00

âFor it happens in this, as the physicians say it happens in hectic fever, that in the beginning of the malady it is easy to cure but difficult to detect,...

NEJM â Collection Updates for Neurologic Disease

CLINICAL PRACTICE: Jet Lag

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 3-2010 â A 5-Month-Old Boy with Developmental Delay and Irritability

REVIEW ARTICLE: Alzheimer's Disease

CORRESPONDENCE: Lovastatin in X-Linked Adrenoleukodystrophy

REVIEW ARTICLE: Nosocomial Bacterial Meningitis

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Gene Therapy for Color Blindness

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 38-2009 â A 16-Year-Old Boy with Paroxysmal Headaches and Visual Changes

IMAGES IN CLINICAL MEDICINE: Blurred Vision after Cardiac Catheterization

CLINICAL PROBLEM-SOLVING: A Crisis in Late Pregnancy

ORIGINAL ARTICLE: A Novel Protective Prion Protein Variant that Colocalizes with Kuru Exposure

INTERACTIVE MEDICAL CASE: A Crisis in Late Pregnancy

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 35-2009 â A 60-Year-Old Male Renal-Transplant Recipient with Renal Insufficiency, Diabetic Ketoacidosis, and Mental-Status Changes

SPECIAL ARTICLE: Outcome Reporting in Industry-Sponsored Trials of Gabapentin for Off-Label Use

ORIGINAL ARTICLE: On-Pump versus Off-Pump Coronary-Artery Bypass Surgery

IMAGES IN CLINICAL MEDICINE: Central Nervous System Hemorrhage

PERSPECTIVE: When Diseases Disappear â The Case of Familial Dysautonomia

IMAGES IN CLINICAL MEDICINE: Keratoconus Complicated by Acute Corneal Hydrops

EDITORIAL: Dying from Dementia

IMAGES IN CLINICAL MEDICINE: Living with One Hemisphere â A Large Porencephalic Cyst

ORIGINAL ARTICLE: The Clinical Course of Advanced Dementia

IMAGES IN CLINICAL MEDICINE: Cerebrospinal Fluid Leak

ORIGINAL ARTICLE: Moderate Hypothermia to Treat Perinatal Asphyxial Encephalopathy

ORIGINAL ARTICLE: Treatment of Medulloblastoma with Hedgehog Pathway Inhibitor GDC-0449

ORIGINAL ARTICLE: Progressive Multifocal Leukoencephalopathy after Natalizumab Monotherapy

ORIGINAL ARTICLE: Treatment of Progressive Multifocal Leukoencephalopathy Associated with Natalizumab

ORIGINAL ARTICLE: Asymptomatic Reactivation of JC Virus in Patients Treated with Natalizumab

PERSPECTIVE: Reemergence of PML in Natalizumab-Treated Patients â New Cases, Same Concerns

CORRESPONDENCE: Renal Sympathetic-Nerve Ablation for Uncontrolled Hypertension

IMAGES IN CLINICAL MEDICINE: Retinal Detachment in Malignant Hypertension

CORRESPONDENCE: Vision 1 Year after Gene Therapy for Leber's Congenital Amaurosis

CORRESPONDENCE: Spinal Injuries in the Sichuan Earthquake

CORRESPONDENCE: Mirror Therapy for Chronic Complex Regional Pain Syndrome Type 1 and Stroke

CLINICAL PRACTICE: Neurologic Prognosis after Cardiac Arrest

CORRESPONDENCE: Whole-Body Magnetic Resonance Neurography

ORIGINAL ARTICLE: AGC1 Deficiency Associated with Global Cerebral Hypomyelination

IMAGES IN CLINICAL MEDICINE: Myotonic Dystrophy

CORRESPONDENCE: Muscle Glycogenosis Due to Phosphoglucomutase 1 Deficiency

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 23-2009 â A 13-Year-Old Boy with Headache, Nausea, Seizures, and Hypertension

ORIGINAL ARTICLE: Hearing Improvement after Bevacizumab in Patients with Neurofibromatosis Type 2

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Memory and the NMDA Receptors

EDITORIAL: Effect of Genetic Testing for Risk of Alzheimer's Disease

ORIGINAL ARTICLE: Longitudinal Modeling of Age-Related Memory Decline and the Îµ4 Effect

ORIGINAL ARTICLE: Disclosure of Genotype for Risk of Alzheimer's Disease

IMAGES IN CLINICAL MEDICINE: Traumatic Bleb

ORIGINAL ARTICLE: Lack of Association between Folate-Receptor Autoantibodies and Neural-Tube Defects

EDITORIAL: Diabetes Complications and the ReninâAngiotensin System

REVIEW ARTICLE: Rhabdomyolysis and Acute Kidney Injury

ORIGINAL ARTICLE: Renal and Retinal Effects of Enalapril and Losartan in Type 1 Diabetes

BOOK REVIEW:

ORIGINAL ARTICLE: Treating Childhood Acute Lymphoblastic Leukemia without Cranial Irradiation

IMAGES IN CLINICAL MEDICINE: Combined-System Disease

BOOK REVIEW:

BOOK REVIEW:

EDITORIAL: Cool with Plaques and Tangles

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 17-2009 â A 30-Year-Old Man with Progressive Neurologic Deficits

ORIGINAL ARTICLE: Age, Neuropathology, and Dementia

IMAGES IN CLINICAL MEDICINE: Patulous Eustachian Tube and Eustachian-Tube Dysfunction

BOOK REVIEW:

IMAGES IN CLINICAL MEDICINE: Bull's-Eye Maculopathy

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 15-2009 â A 25-Year-Old Man with Coma after Cardiac Arrest

ORIGINAL ARTICLE: Fatal Case of Deer Tick Virus Encephalitis

ORIGINAL ARTICLE: Antivenom for Critically Ill Children with Neurotoxicity from Scorpion Stings

BOOK REVIEW:

BOOK REVIEW:

EDITORIAL: Membrane Physiology â Bridging the Gap between Medical Disciplines

CLINICAL PROBLEM-SOLVING: A Red Flag

ORIGINAL ARTICLE: Epilepsy, Ataxia, Sensorineural Deafness, Tubulopathy, and Mutations

IMAGES IN CLINICAL MEDICINE: Chvostek's Sign and Carpopedal Spasm

IMAGES IN CLINICAL MEDICINE: Bilateral Subacute Subdural Hematomas

CORRESPONDENCE: Mutations in a Thiamine-Transporter Gene and Wernicke's-like Encephalopathy

ORIGINAL ARTICLE: Association of HTRA1 Mutations and Familial Ischemic Cerebral Small-Vessel Disease

EDITORIAL: Which Drug for the Pregnant Woman with Epilepsy?

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 12-2009 â A 46-Year-Old Man with Migraine, Aphasia, and Hemiparesis and Similarly Affected Family Members

IMAGES IN CLINICAL MEDICINE: Keyhole Pupils

ORIGINAL ARTICLE: Cognitive Function at 3 Years of Age after Fetal Exposure to Antiepileptic Drugs

PERSPECTIVE: Care of War Veterans with Mild Traumatic Brain Injury â Flawed Perspectives

BOOK REVIEW:

BOOK REVIEW:

BOOK REVIEW:

NEJM â Collection Updates for Neurologic Disease

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 3-2010 â A 5-Month-Old Boy with Developmental Delay and Irritability

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 38-2009 â A 16-Year-Old Boy with Paroxysmal Headaches and Visual Changes

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 35-2009 â A 60-Year-Old Male Renal-Transplant Recipient with Renal Insufficiency, Diabetic Ketoacidosis, and Mental-Status Changes

PERSPECTIVE: When Diseases Disappear â The Case of Familial Dysautonomia

IMAGES IN CLINICAL MEDICINE: Living with One Hemisphere â A Large Porencephalic Cyst

PERSPECTIVE: Reemergence of PML in Natalizumab-Treated Patients â New Cases, Same Concerns

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 23-2009 â A 13-Year-Old Boy with Headache, Nausea, Seizures, and Hypertension

EDITORIAL: Diabetes Complications and the ReninâAngiotensin System

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 17-2009 â A 30-Year-Old Man with Progressive Neurologic Deficits

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 15-2009 â A 25-Year-Old Man with Coma after Cardiac Arrest

EDITORIAL: Membrane Physiology â Bridging the Gap between Medical Disciplines

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 12-2009 â A 46-Year-Old Man with Migraine, Aphasia, and Hemiparesis and Similarly Affected Family Members

PERSPECTIVE: Care of War Veterans with Mild Traumatic Brain Injury â Flawed Perspectives

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 8-2009 â A 36-Year-Old Woman with Headache, Hypertension, and Seizure 2 Weeks Post Partum

CLINICAL DECISIONS: Care of an Unresponsive Patient with a Poor Prognosis â Polling Results

PERSPECTIVE: Serotonin Rising â The Bone, Brain, Bowel Connection

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 6-2009 â A 37-Year-Old Woman with Vertigo, Facial Weakness, and a Generalized Seizure

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 5-2009 â A 47-Year-Old Woman with a Rash and Numbness and Pain in the Legs

CORRESPONDENCE: Prevalence of Mitochondrial 1555AâG Mutation in Adults of European Descent

CORRESPONDENCE: Prevalence of Mitochondrial 1555AâG Mutation in European Children

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 1-2009 â A 57-Year-Old Man with Progressive Cognitive Decline

CORRESPONDENCE: Persistent Fainting after Implantation of a âCurativeâ Pacemaker

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 40-2008 â A 26-Year-Old Man with Blurred Vision

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 37-2008 â A 17-Year-Old Boy with a Pituitary Tumor and Skull Abnormalities

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 36-2008 â A 59-Year-Old Man with Chronic Daily Headache

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 35-2008 â A 65-Year-Old Man with Confusion and Memory Loss

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 35-2008 â A 65-Year-Old Man with Confusion and Memory Loss

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 35-2008 â A 65-Year-Old Man with Confusion and Memory Loss

ORIGINAL ARTICLE: Subthalamic Nucleus Stimulation in Severe ObsessiveâCompulsive Disorder

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 34-2008 â A 58-Year-Old Woman with Neck Pain and Fever

EDITORIAL: Cytogenetic Technology â Genotype and Phenotype

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL: Case 28-2008 â An 8-Day-Old Infant with Congenital Deafness, Lethargy, and Hypothermia

CLINICAL IMPLICATIONS OF BASIC RESEARCH: Targeting Intraocular Neovascularization and Edema â One Drop at a Time