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Clinical Implications of Basic Neuroscience Research 
Kristopher T. Kahle, MD, PhD; Arjun Khanna, BS; David E. Clapham, MD, PhD; Clifford J. Woolf, MD, PhD

Peripheral neuropathic pain, typified by the development of spontaneous pain or pain hypersensitivity following injury to the peripheral nervous system, is common, greatly impairs quality of life, and is inadequately treated with available drugs. Maladaptive changes in chloride homeostasis due to a decrease in the functional expression of ...

Editorial 
Bradley R. Foerster, MD, PhD; Eva L. Feldman, MD, PhD
Amyotrophic lateral sclerosis (ALS) is a clinically heterogeneous disease and presents with varying degrees of upper motor and lower motor neuron involvement. In certain cases, diagnosis can be challenging even for an experienced neurologist. Electrophysiology supplements the physical examination for detection of lower motor neuron involvement and is combined with ...
Images in Neurology 
Benjamin Gory, MD, MSc; Thomas Ritzenthaler, MD, MSc; Roberto Riva, MD; Norbert Nighoghossian, MD, PhD; Francis Turjman, MD, PhD
Diffusion-weighted imaging (DWI) has revealed the reversal of often sizeable damage due to brainstem lesions in patients treated within 4.5 hours, and this reversibility of damage was strongly associated with early neurological improvement.1 In posterior circulation stroke, the reversibility of damage due to a DWI-detected lesion is poorly documented after ...
Original Investigation 
Koen Van Laere, MD, PhD, DSc; Annelies Vanhee, MD; Jolien Verschueren, MD; Liesbeth De Coster, MD; An Driesen, MD; Patrick Dupont, PhD; Wim Robberecht, MD, PhD; Philip Van Damme, MD, PhD
Includes: Supplemental Content

Importance  Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder primarily affecting the motor system, with extramotor involvement to a variable extent. Biomarkers for early differential diagnosis and prognosis are needed. An autosomal dominant hexanucleotide (GGGGCC) expansion in the noncoding region of the chromosome 9 open reading frame ...

Case Report/Case Series 
Michael C. Kruer, MD; Romana Hoeftberger, MD; Kit Yeng Lim, MD; Jason C. Coryell, MD; Melissa D. Svoboda, MD; Randall L. Woltjer, MD, PhD; Josep Dalmau, MD, PhD

Importance  Autoantibodies to the γ-aminobutyric acid type B (GABAB) receptor have recently been identified as a cause of autoimmune encephalitis. Most patients with GABAB encephalitis have presented with limbic encephalitis. About half of the cases reported have been paraneoplastic in origin, with the majority ...

Original Investigation 
Wendy K. M. Liew, MBChB, MRCPCH; Christine A. Powell, MA; Steven R. Sloan, MD, PhD; Robert C. Shamberger, MD; Christopher B. Weldon, MD, PhD; Basil T. Darras, MD; Peter B. Kang, MD
Includes: Supplemental Content

Importance  Juvenile myasthenia gravis (MG) is a relatively rare autoimmune disorder. The comparative efficacy of plasmapheresis (PLEX) vs immunoglobulin as maintenance therapy is unclear for this childhood disease.

Objective  To determine whether PLEX or intravenous immunoglobulin (IVIG) therapy is more effective as maintenance therapy in this ...

Review 
Ain A. Neuhaus, BA; Tamer Rabie, PhD; Brad A. Sutherland, PhD; Michalis Papadakis, PhD; Gina Hadley, MRCP; Ruiyao Cai, BSc; Alastair M. Buchan, FMedSci

Importance  Preclinical stroke research has had a remarkably low translational success rate, and the clinical need for novel neuroprotective therapeutics has gone largely unmet, especially in light of the severe underuse of thrombolysis in acute ischemic stroke.

Objective  In this review, we aim to provide a ...

Viewpoint 
P. Justin Rossi, BA; Andre Machado, MD, PhD; Michael S. Okun, MD
Medicare coverage plays an important role in innovative device-based therapy in a market with increasing medical device costs and scarce financial resources. In January 2014, the Centers for Medicare and Medicaid Services (CMS) implemented changes to coverage policies for items and services associated with investigational device exemption (IDE) studies.1 Medicare’s ...
Observation  FREE
David Y. Johnson, MD; Diana L. Dunkelberger, MA; Maya Henry, PhD; Aissatou Haman, MD; Michael D. Greicius, MD, PhD; Katherine Wong, BA; Stephen J. DeArmond, MD, PhD; Bruce L. Miller, MD; Maria Luisa Gorno-Tempini, MD, PhD; Michael D. Geschwind, MD, PhD

Objective To report the clinical, neuropsychological, linguistic, imaging, and neuropathological features of a unique case of sporadic Jakob-Creutzfeldt disease in which the patient presented with a logopenic variant of primary progressive aphasia.

Design Case report.

Setting Large referral center for atypical memory and aging disorders, particularly Jakob-Creutzfeldt disease.

...
In this issue of Archives, McKeon et al1 report that 10 of 81 patients with stiff-man syndrome from the Mayo Clinic in Rochester, Minnesota, had serum and/or cerebrospinal fluid IgG antibodies that bound to fixed, permeabilized HEK293 cells (an immortalized human kidney line) that had been transfected to express the ...
Clinical Pathologic Conference  FREE
John J. Konikkara, MD; Osvaldo H. Perurena, MD; Steven Warach, MD, PhD; Steven C. Bauserman, MD

A 62-year-old man with no significant medical history experienced fatigue, night sweats, hoarseness of voice, and dry cough, which were followed by vision disturbances in his left eye. He lost about 4.5 kg (10 lb) in just over a month. Three weeks later, he had difficulty recollecting his ...

Observation  FREE
Hans-Christian Hansen, MD; Christine Klingbeil; Josep Dalmau, MD, PhD; Wenhan Li, MD; Benedikt Weißbrich, MD; Klaus-Peter Wandinger, MD

Background Anti– N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe autoimmune disorder characterized by high intrathecal antibody synthesis. Little is known about the long-term follow-up of the cerebrospinal fluid antibody status.

Objective To describe persistent intrathecal antibody synthesis in a clinically healthy person 15 years after ...

Original Contribution  FREE
Sara E. Hocker, MD; Jeffrey W. Britton, MD; Jayawant N. Mandrekar, PhD; E. F. M. Wijdicks, MD, PhD; Alejandro A. Rabinstein, MD
Includes: Supplemental Content

Objective To further characterize the demographics, outcomes, and prognostic factors for refractory status epilepticus (RSE).

Design Retrospective analysis of all the episodes of RSE treated between January 1, 1999, and August 30, 2011.

Setting Neurointensive care unit within a tertiary referral center, Mayo Clinic, Rochester, Minnesota.

Patients Refractory ...

Original Contribution  FREE
Rita João Guerreiro, PhD; Ebba Lohmann, MD; José Miguel Brás, PhD; Jesse Raphael Gibbs, MS; Jonathan D. Rohrer, MRCP; Nicole Gurunlian, MS; Burcu Dursun, MD; Basar Bilgic, MD; Hasmet Hanagasi, MD; Hakan Gurvit, MD; Murat Emre, MD; Andrew Singleton, PhD; John Hardy, PhD
Includes: Supplemental Content

Objective To identify new genes and risk factors associated with frontotemporal dementia (FTD). Several genes and loci have been associated with different forms of FTD, but a large number of families with dementia do not harbor mutations in these genes.

Design Whole-exome sequencing and whole-genome genotyping were performed ...

Original Contribution  FREE
Janey Prodoehl, PhD; Peggy J. Planetta, PhD; Ajay S. Kurani, MS; Cynthia L. Comella, MD; Daniel M. Corcos, PhD; David E. Vaillancourt, PhD

Objective To compare differences in functional brain activity between tremor- and nontremor-dominant subtypes of Parkinson disease (PD) using functional magnetic resonance imaging.

Design In our study, patients with tremor-dominant PD and those with nontremor-dominant PD performed a grip task, and the results obtained were compared using voxelwise analysis. ...

The treatment of relapsing multiple sclerosis (MS) remains a rare beacon of success in the long campaign against neurological disease. The ready availability of a phase II clinical outcome (eg, gadolinium-enhancing lesion) that tracks a relevant phase III clinical outcome (eg, annualized relapse rate) is one of the major reasons ...
Neurological Review  FREE
Hao Deng, MD, PhD; Hui Liang, MD; Joseph Jankovic, MD

Parkinson disease is one of the most common neurodegenerative diseases associated with aging. At least 18 genetic loci and 13 disease-related genes for parkinsonism have been identified. Among them, PARK15 -associated parkinsonism, also referred to as parkinsonian-pyramidal disease (PPD), was found to be caused by mutations in the ...

Original Contribution  FREE
Shiv Saidha, MBBCh, MRCPI; Elias S. Sotirchos, MD; Jiwon Oh, MD, FRCPC; Stephanie B. Syc, ScB; Michaela A. Seigo, ScB; Navid Shiee, MS; Chistopher Eckstein, MD; Mary K. Durbin, PhD; Jonathan D. Oakley, PhD; Scott A. Meyer, PhD; Teresa C. Frohman, BS; Scott Newsome, DO; John N. Ratchford, MD; Laura J. Balcer, MD, MSCE; Dzung L. Pham, PhD; Ciprian M. Crainiceanu, PhD; Elliot M. Frohman, MD, PhD; Daniel S. Reich, MD, PhD; Peter A. Calabresi, MD
Includes: Supplemental Content

Objective To determine the relationships between conventional and segmentation-derived optical coherence tomography (OCT) retinal layer thickness measures with intracranial volume (a surrogate of head size) and brain substructure volumes in multiple sclerosis (MS).

Design Cross-sectional study.

Setting Johns Hopkins University, Baltimore, Maryland.

Participants A total of 84 patients ...

Observation  FREE
Viviane Chalhoub, MD; Lise Abi-Rafeh, MD; Kamal Hachem, MD; Eliane Ayoub, MD; Patricia Yazbeck, MD

Objective To highlight the possible association of intracranial aneurysm with autosomal recessive polycystic kidney disease.

Design, Setting, and Patient To our knowledge, this association has been reported only twice in the medical literature. We herein report the case of a 21-year-old man with autosomal recessive polycystic kidney disease, ...

Original Contribution  FREE
Ross W. Paterson, MRCP; Charles C. Torres-Chae, MPA; Amy L. Kuo, MS, RN, GNP; Tim Ando, BA; Elizabeth A. Nguyen, BS; Katherine Wong, BS; Stephen J. DeArmond, MD, PhD; Aissa Haman, MD; Paul Garcia, MD; David Y. Johnson; Bruce L. Miller, MD; Michael D. Geschwind, MD, PhD

Objectives To identify the misdiagnoses of patients with sporadic Jakob-Creutzfeldt disease (sCJD) during the course of their disease and determine which medical specialties saw patients with sCJD prior to the correct diagnosis being made and at what point in the disease course a correct diagnosis was made.

Design...

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