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June 2019 - July 1959

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June 2016, Vol 73, No. 6, Pages 613-763

In This Issue of JAMA Neurology

Highlights

Abstract Full Text
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JAMA Neurol. 2016;73(6):613. doi:10.1001/jamaneurol.2015.2461
Viewpoint

Next Generation Neurology

Neurologist Comfort in the Use of Next-Generation Sequencing Diagnostics: Current State and Future Prospects

Abstract Full Text
JAMA Neurol. 2016;73(6):621-622. doi:10.1001/jamaneurol.2016.0168

This Viewpoint assesses the frequency of next-generation sequencing use among neurologists and identifies barriers in its implementation.

Editorial

Precision Medicine for Charcot-Marie-Tooth Disease

Abstract Full Text
JAMA Neurol. 2016;73(6):623-624. doi:10.1001/jamaneurol.2016.0285

Treatment for Refractory Myasthenia Gravis—New Lymphs for Old

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JAMA Neurol. 2016;73(6):624-626. doi:10.1001/jamaneurol.2016.0191

Understanding Atomic Interactions to Achieve Well-being

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JAMA Neurol. 2016;73(6):626-627. doi:10.1001/jamaneurol.2016.0546

Pain Perception: Multiple Matrices or One?

Abstract Full Text
JAMA Neurol. 2016;73(6):628-630. doi:10.1001/jamaneurol.2016.0757

Mitochondrial Extrapyramidal Syndromes: Using Age and Phenomenology to Guide Genetic Testing

Abstract Full Text
JAMA Neurol. 2016;73(6):630-632. doi:10.1001/jamaneurol.2016.0756

Cerebral Microbleeds and Thrombolysis: Clinical Consequences and Mechanistic Implications

Abstract Full Text
JAMA Neurol. 2016;73(6):632-635. doi:10.1001/jamaneurol.2016.0576

Understanding Small Fiber Neuropathy: The Long and Short of It

Abstract Full Text
JAMA Neurol. 2016;73(6):635-637. doi:10.1001/jamaneurol.2016.0256

Alemtuzumab and Multiple Sclerosis: Another Note of Caution

Abstract Full Text
JAMA Neurol. 2016;73(6):637-638. doi:10.1001/jamaneurol.2016.0259

New Genetic Links Between Alzheimer Disease and Immune-Mediated Diseases Revealed by Overlapping Genome-wide Association Study Hits

Abstract Full Text
JAMA Neurol. 2016;73(6):638-640. doi:10.1001/jamaneurol.2016.0407

Cognitive Decline in Preclinical Stage 2 Alzheimer Disease and Implications for Prevention Trials

Abstract Full Text
JAMA Neurol. 2016;73(6):640-642. doi:10.1001/jamaneurol.2016.0281

The Association Between Atrophy and the Prognosis of Anti-N-Methyl-d-Aspartate Receptor Encephalitis

Abstract Full Text
JAMA Neurol. 2016;73(6):643-644. doi:10.1001/jamaneurol.2016.0530
Original Investigation

Phenotypic Variability of Childhood Charcot-Marie-Tooth Disease

Abstract Full Text
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JAMA Neurol. 2016;73(6):645-651. doi:10.1001/jamaneurol.2016.0171

This cross-sectional study assesses the variability of disease severity in a large cohort of children and adolescents with Charcot-Marie-Tooth disease.

Myasthenia Gravis Treated With Autologous Hematopoietic Stem Cell Transplantation

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JAMA Neurol. 2016;73(6):652-658. doi:10.1001/jamaneurol.2016.0113

This study reports 7 cases of severe myasthenia gravis treated with autologous hematopoietic stem cell transplant in which consistent, durable, symptom-free, and treatment-free remission was achieved.

Pharmacotherapy for Pain in a Family With Inherited Erythromelalgia Guided by Genomic Analysis and Functional Profiling

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JAMA Neurol. 2016;73(6):659-667. doi:10.1001/jamaneurol.2016.0389

This double-blind, cross-over study of carbamazepine in 2 patients with inherited erythromelalgia aims to determine whether pain can be attenuated via pharmacotherapy guided by genomic analysis and functional profiling.

Clinical, Genetic, and Radiological Features of Extrapyramidal Movement Disorders in Mitochondrial Disease

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JAMA Neurol. 2016;73(6):668-674. doi:10.1001/jamaneurol.2016.0355

This cohort study describes the phenotype, genetic etiology, and investigation of extrapyramidal movement disorders in pediatric and adult patients with mitochondrial disease.

Risk of Symptomatic Intracerebral Hemorrhage After Intravenous Thrombolysis in Patients With Acute Ischemic Stroke and High Cerebral Microbleed Burden: A Meta-analysis

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JAMA Neurol. 2016;73(6):675-683. doi:10.1001/jamaneurol.2016.0292

This meta-analysis investigates the association of high cerebral microbleed burden with the risk of symptomatic intracerebral hemorrhage in patients with acute ischemic stroke treated with intravenous thrombolysis.

Longitudinal Assessment of Small Fiber Neuropathy: Evidence of a Non–Length-Dependent Distal Axonopathy

Abstract Full Text
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JAMA Neurol. 2016;73(6):684-690. doi:10.1001/jamaneurol.2016.0057

This case-control study compares the longitudinal rate and pattern of intraepidermal nerve fiber density change in idiopathic small fiber neuropathy (SFN), impaired glucose tolerance–associated SFN, and diabetes mellitus–associated SFN.

Association Between Genetic Traits for Immune-Mediated Diseases and Alzheimer Disease

Abstract Full Text
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JAMA Neurol. 2016;73(6):691-697. doi:10.1001/jamaneurol.2016.0150

This genetic epidemiology study examines whether there is an association between genetic traits for immune-mediated diseases that promote inflammation and Alzheimer disease.

Hypothetical Preclinical Alzheimer Disease Groups and Longitudinal Cognitive Change

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JAMA Neurol. 2016;73(6):698-705. doi:10.1001/jamaneurol.2016.0194

This cohort study of 4 hypothetical preclinical Alzheimer disease groups identifies which group of cognitively normal individuals shows the greatest cognitive decline over time based on their cerebrospinal fluid biomarker profile.

Association of Progressive Cerebellar Atrophy With Long-term Outcome in Patients With Anti-N-Methyl-d-Aspartate Receptor Encephalitis

Abstract Full Text
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JAMA Neurol. 2016;73(6):706-713. doi:10.1001/jamaneurol.2016.0232

This observational study examines long-term outcomes in patients with anti-N-methyl-d-aspartate receptor encephalitis.

Clinical and Immunologic Investigations in Patients With Stiff-Person Spectrum Disorder

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JAMA Neurol. 2016;73(6):714-720. doi:10.1001/jamaneurol.2016.0133

This case-series study reports the clinical and immunologic findings in a cohort of patients with stiff-person spectrum disorder, including stiff-person syndrome, stiff-limb syndrome, or stiff-person syndrome plus progressive encephalomyelitis with rigidity, myoclonus, or other symptoms.

Association Between Anticholinergic Medication Use and Cognition, Brain Metabolism, and Brain Atrophy in Cognitively Normal Older Adults

Abstract Full Text
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JAMA Neurol. 2016;73(6):721-732. doi:10.1001/jamaneurol.2016.0580

This study assesses the association between anticholinergic medication use and cognition, glucose metabolism, and brain atrophy in cognitively normal older adults from the Alzheimer’s Disease Neuroimaging Initiative and the Indiana Memory and Aging Study.

Features of Patients With Nonfluent/Agrammatic Primary Progressive Aphasia With Underlying Progressive Supranuclear Palsy Pathology or Corticobasal Degeneration

Abstract Full Text
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JAMA Neurol. 2016;73(6):733-742. doi:10.1001/jamaneurol.2016.0412

This clinical-pathological study characterizes the neurological, cognitive, and neuroimaging features of patients with nonfluent/agrammatic primary progressive aphasia—in whom either progressive supranuclear palsy or corticobasal degeneration was eventually confirmed at autopsy—at initial presentation and at 1-year follow-up and identifies features associated with underlying pathology.

Review

A Clinical Approach to the Diagnosis of Traumatic Encephalopathy Syndrome: A Review

Abstract Full Text
JAMA Neurol. 2016;73(6):743-749. doi:10.1001/jamaneurol.2015.5015

This narrative review assesses a proposed new diagnostic construct for the clinical syndrome associated with repetitive exposure to head impact trauma to provide a clinical framework for the diagnosis of traumatic encephalopathy syndrome.

Images in Neurology

Imaging Active Intracerebral Hemorrhage After Attempted Endovascular Thrombectomy

Abstract Full Text
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JAMA Neurol. 2016;73(6):754. doi:10.1001/jamaneurol.2016.0570

This case report describes a woman in her 70s with a history of atrial fibrillation who presented with slurred speech and difficulty ambulating for 1 hour.

JAMA Neurology Clinical Challenge

Keeping an Eye on This Headache

Abstract Full Text
JAMA Neurol. 2016;73(6):751-752. doi:10.1001/jamaneurol.2016.0346

A 24-year-old woman had a strong unilateral headache located in her left cheek, eye, and forehead that evolved during the evening hours, peaked at night, and decreased again until the morning, without any remarkable trigger along with ipsilateral lacrimation, a red eye, and periorbital swelling but without ptosis. What is your diagnosis?

Research Letter

The “Pain Matrix” in Pain-Free Individuals

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JAMA Neurol. 2016;73(6):755-756. doi:10.1001/jamaneurol.2016.0653

This study administered noxious mechanical stimuli to individuals with congenital insensitivity to pain and sampled their brain activity with functional magnetic resonance imaging.

Observation

Fulminant Central Nervous System Nocardiosis in a Patient Treated With Alemtuzumab for Relapsing-Remitting Multiple Sclerosis

Abstract Full Text
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JAMA Neurol. 2016;73(6):757-759. doi:10.1001/jamaneurol.2016.0146

This case report describes a case of fulminant central nervous system nocardiosis at 5 months after initiation of alemtuzumab in a patient with multiple sclerosis.

Comment & Response

Hemodynamics in Symptomatic Atherosclerotic Vertebrobasilar Occlusive Disease

Abstract Full Text
JAMA Neurol. 2016;73(6):759. doi:10.1001/jamaneurol.2016.0524

Hemodynamics in Symptomatic Atherosclerotic Vertebrobasilar Occlusive Disease—Reply

Abstract Full Text
JAMA Neurol. 2016;73(6):759-760. doi:10.1001/jamaneurol.2016.0527

American Academy of Neurology Guidelines and the Neurologic Determination of Death

Abstract Full Text
JAMA Neurol. 2016;73(6):760-761. doi:10.1001/jamaneurol.2016.0428

Olfactory Loss—On the Road to Potential Diagnosis Criteria of Alzheimer Disease

Abstract Full Text
JAMA Neurol. 2016;73(6):761-762. doi:10.1001/jamaneurol.2016.0326

American Academy of Neurology Guidelines and the Neurologic Determination of Death—Reply

Abstract Full Text
JAMA Neurol. 2016;73(6):761. doi:10.1001/jamaneurol.2016.0431

Association Between Vitamin D Status and Cognitive Decline

Abstract Full Text
JAMA Neurol. 2016;73(6):762. doi:10.1001/jamaneurol.2016.0553
Correction

Error in Author Degree

Abstract Full Text
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JAMA Neurol. 2016;73(6):762. doi:10.1001/jamaneurol.2016.1392
Book and Media Review

Review of Merritt’s Neurology, 13th ed

Abstract Full Text
JAMA Neurol. 2016;73(6):763. doi:10.1001/jamaneurol.2016.1005
JAMA Neurology Masthead

JAMA Neurology

Abstract Full Text
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JAMA Neurol. 2016;73(6):620. doi:10.1001/jamaneurol.2015.2462
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