Neurology




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Limited Benefits Halt Enrollment in Hematopoietic Stem Cell Transplantation Trial for Stiff-Person Syndrome

Marinos C. Dalakas

doi : 10.1212/WNL.0000000000011349

February 09, 2021; 96 (6) 

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From COVID-19 to ALS

Jeffrey C. McClean, James A. Russell

doi : 10.1212/WNL.0000000000011441

February 09, 2021; 96 (6) 

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Preservation of Memory in Alzheimer-Related Primary Progressive Aphasia

Seyed Ahmad Sajjadi, Sharon Ash, Stefano Cappa

doi : 10.1212/WNL.0000000000011404

February 09, 2021; 96 (6) 

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Autologous Hematopoietic Stem Cell Transplantation for Stiff-Person Spectrum Disorder

Richard K. Burt, Roumen Balabanov, Xiaoqiang Han, Kathleen Quigley, Indira Arnautovic, Irene Helenowski, John Rose, Teepu Siddique

doi : 10.1212/WNL.0000000000011338

February 09, 2021; 96 (6) 

Objective To test the hypothesis that autologous nonmyeloablative hematopoietic stem cell transplantation (HSCT) is safe and shows efficacy in the treatment of stiff-person spectrum disorder (SPSD).

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Association of Hospital-Diagnosed Infections and Antibiotic Use With Risk of Developing Guillain-Barré Syndrome

Lotte S. Levison,  View ORCID ProfileReimar W. Thomsen,  View ORCID ProfileS?ren H. Sindrup,  View ORCID ProfileHenning Andersen

doi : 10.1212/WNL.0000000000011342

February 09, 2021; 96 (6) 

Objective To determine whether hospital-diagnosed and community-treated infections are important Guillain-Barré syndrome (GBS) risk factors, we investigated the magnitude and duration of associated GBS risk.

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Comparison of Phenotypic Characteristics and Prognosis Between Black and White Patients in a Tertiary ALS Clinic

David Brand, Meraida Polak, Jonathan D. Glass, Christina N. Fournier

doi : 10.1212/WNL.0000000000011396

February 09, 2021; 96 (6) 

Objective To compare characteristics between Black and White patients with amyotrophic lateral sclerosis (ALS) in order to identify disparities and phenotypic variability.

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Correlates of Fatigability in Patients With Spinal Muscular Atrophy

 View ORCID ProfileBart Bartels, Janke F. de Groot, Laura E. Habets,  View ORCID ProfileRenske I. Wadman, Fay-Lynn Asselman, Edward E.S. Nieuwenhuis,  View ORCID ProfileRuben P.A. van Eijk, H. Stephan Goedee,  View ORCID ProfileW. Ludo van der Pol

doi : 10.1212/WNL.0000000000011230

February 09, 2021; 96 (6) 

Objective To determine the associations between fatigability and muscle strength, motor function, neuromuscular junction (NMJ) function, and perceived fatigue in spinal muscular atrophy (SMA), we assessed 61 patients with SMA.

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Efficacy and Safety of Rozanolixizumab in Moderate to Severe Generalized Myasthenia Gravis

Vera Bril, Michael Benatar, Henning Andersen, John Vissing, Melissa Brock, Bernhard Greve, Peter Kiessling, Franz Woltering, Laura Griffin, Peter Van den Bergh, on behalf of the MG0002 Investigators

doi : 10.1212/WNL.0000000000011108

February 09, 2021; 96 (6) 

Objective To explore the clinical efficacy and safety of subcutaneous (SC) rozanolixizumab, an anti-neonatal Fc receptor humanized monoclonal antibody, in patients with generalized myasthenia gravis (gMG).

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Cranial Nerve Disorders Associated With Immune Checkpoint Inhibitors

 View ORCID ProfileAlberto Vogrig, Sergio Mu?iz-Castrillo,  View ORCID ProfileBastien Joubert,  View ORCID ProfileGéraldine Picard,  View ORCID ProfileVéronique Rogemond, François Skowron, Madalina Egri,  View ORCID ProfileVirginie Desestret,  View ORCID ProfileCaroline Tilikete,  View ORCID ProfileDimitri Psimaras,  View ORCID ProfileFrançois Ducray, Jérôme Honnorat

doi : 10.1212/WNL.0000000000011340

February 09, 2021; 96 (6) 

Objective To describe the spectrum, treatment, and outcome of cranial nerve disorders associated with immune checkpoint inhibitor (Cn-ICI).

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Neurogenic Dysphagia

Tobias Warnecke, Bendix Labeit, Jens Schroeder, Alexander Reckels, Sigrid Ahring, Sriramya Lapa, Inga Claus, Paul Muhle, Sonja Suntrup-Krueger, Rainer Dziewas

doi : 10.1212/WNL.0000000000011350

February 09, 2021; 96 (6) 

Objective Introduction and validation of a phenotypic classification of neurogenic dysphagia based on flexible endoscopic evaluation of swallowing (FEES).

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Association of Dilated Perivascular Spaces and Disease Severity in Patients With Huntington Disease

Suk Tak Chan, Nathaniel D. Mercaldo, Bernard Ravina, Steven M. Hersch, Herminia D. Rosas

doi : 10.1212/WNL.0000000000011121

February 09, 2021; 96 (6) 

Objective To quantify the percent volume of dilated perivascular space (PVS) in the subcortical forebrain in patients with early Huntington disease (HD) and to explore the relationship between PVS and disease severity.

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Dietary Antioxidants and the Risk of Parkinson Disease

Essi Hantikainen, Ylva Trolle Lagerros, Weimin Ye, Mauro Serafini, Hans-Olov Adami, Rino Bellocco, Stephanie Bonn

doi : 10.1212/WNL.0000000000011373

February 09, 2021; 96 (6) 

Objective To determine whether high baseline dietary antioxidants and total nonenzymatic antioxidant capacity (NEAC) is associated with a lower risk of Parkinson disease (PD) in men and women, we prospectively studied 43,865 men and women from a large Swedish cohort.

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Association of Hippocampal Subfields, CSF Biomarkers, and Cognition in Patients With Parkinson Disease Without Dementia

 View ORCID ProfileSara Becker, Oliver Granert, Maarten Timmers,  View ORCID ProfileAndrea Pilotto, Luc Van Nueten, Benjamin Roeben, Giacomo Salvadore, Wendy R. Galpern, Johannes Streffer, Klaus Scheffler, Walter Maetzler, Daniela Berg, Inga Liepelt-Scarfone

doi : 10.1212/WNL.0000000000011224

February 09, 2021; 96 (6) 

Objective To examine whether hippocampal volume loss is primarily associated with cognitive status or pathologic ?-amyloid 1–42 (A?42) levels, this study compared hippocampal subfield volumes between patients with Parkinson disease (PD) with mild cognitive impairment (PD-MCI) and without cognitive impairment (PD-CN) and between patients with low and high A?42 levels, in addition exploring the relationship among hippocampal subfield volumes, CSF biomarkers (A?42, phosphorylated and total tau), neuropsychological tests, and activities of daily living.

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Memory Resilience in Alzheimer Disease With Primary Progressive Aphasia

M.-Marsel Mesulam,  View ORCID ProfileChristina Coventry, Alan Kuang, Eileen H. Bigio, Qinwen Mao, Margaret E. Flanagan, Tamar Gefen, Jaiashre Sridhar, Changiz Geula, Hui Zhang, Sandra Weintraub, Emily J. Rogalski

doi : 10.1212/WNL.0000000000011397

February 09, 2021; 96 (6) 

Objective To determine whether memory is preserved longitudinally in primary progressive aphasia (PPA) associated with Alzheimer disease (AD) and to identify potential factors that maintain memory despite underlying neurofibrillary degeneration of mediotemporal memory areas.

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Association of Ventricular Arrhythmias With Dementia

View ORCID ProfileFaye L. Norby,  View ORCID ProfileAlvaro Alonso,  View ORCID ProfileMary R. Rooney, Ankit Maheshwari, Ryan J. Koene,  View ORCID ProfileMichael Zhang, Elsayed Z. Soliman, Laura R. Loehr, Thomas Mosley, Rebecca F. Gottesman, Josef Coresh, Lin Y. Chen

doi : 10.1212/WNL.0000000000011122

February 09, 2021; 96 (6) 

Objective We performed a cross-sectional analysis to determine whether nonsustained ventricular tachycardia (NSVT) and premature ventricular contractions (PVCs) were associated with dementia in a population-based study.

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Endothelial and Leukocyte-Derived Microvesicles and Cardiovascular Risk After Stroke

Shufan Huo, Nicolle Kr?nkel, Alexander Heinrich Nave,  View ORCID ProfilePia Sophie Sperber,  View ORCID ProfileJessica Lee Rohmann,  View ORCID ProfileSophie K?the Piper, Peter Ulrich Heuschmann,  View ORCID ProfileUlf Landmesser, Matthias Endres,  View ORCID ProfileBob Siegerink, Thomas Günter Liman

doi : 10.1212/WNL.0000000000011223

February 09, 2021; 96 (6) 

Objective To determine the role of circulating microvesicles (MV) on long-term cardiovascular outcomes after stroke, we measured them in patients with first-ever stroke with a 3-year follow-up.

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ANGPTL6 Genetic Variants Are an Underlying Cause of Familial Intracranial Aneurysms

View ORCID ProfileIsabel C. Hostettler,  View ORCID ProfileBenjamin O'Callaghan,  View ORCID ProfileEnrico Bugiardini, Emer O'Connor, Jana Vandrovcova,  View ORCID ProfileIndran Davagnanam, Varinder Alg, Stephen Bonner,  View ORCID ProfileDaniel Walsh,  View ORCID ProfileDiederik Bulters, Neil Kitchen, Martin M. Brown, Joan Grieve, David J. Werring, Henry Houlden, on behalf of the Genetics and Observational Subarachnoid Haemorrhage (GOSH) study investigators

doi : 10.1212/WNL.0000000000011125

February 09, 2021; 96 (6) 

Purpose To understand the role of the angiopoietin-like 6 gene (ANGPTL6) in intracranial aneurysms (IAs), we investigated its role in a large cohort of familial IAs.

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What's Happening in Neurology® Clinical Practice

doi : 10.1212/WNL.0000000000011402

February 09, 2021; 96 (6) 

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What's Happening in Neurology® Neuroimmunology & Neuroinflammation

doi : 10.1212/WNL.0000000000011398

February 09, 2021; 96 (6) 

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What's Happening in Innovations in Care Delivery

doi : 10.1212/WNL.0000000000011399

February 09, 2021; 96 (6) 

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Clinical Practice Guidelines by the Infectious Diseases Society of America, American Academy of Neurology, and American College of Rheumatology

Paul M. Lantos, Jeffrey Rumbaugh, Linda K. Bockenstedt, Yngve T. Falck-Ytter, Maria E. Aguero-Rosenfeld, Paul G. Auwaerter, Kelly Baldwin, Raveendhara R. Bannuru, Kiran K. Belani, William R. Bowie, John A. Branda, David B. Clifford, Francis J. DiMario, John J. Halperin, Peter J. Krause, Valery Lavergne, Matthew H. Liang, H. Cody Meissner, Lise E. Nigrovic, James (Jay) J. Nocton, Mikala C. Osani, Amy A. Pruitt, Jane Rips, Lynda E. Rosenfeld, Margot L. Savoy, Sunil K. Sood, Allen C. Steere, Franc Strle, Robert Sundel, Jean Tsao, Elizaveta E. Vaysbrot, Gary P. Wormser, Lawrence S. Zemel

doi : 10.1212/WNL.0000000000011151

February 09, 2021; 96 (6) 

This evidence-based clinical practice guideline for the prevention, diagnosis, and treatment of Lyme disease was developed by a multidisciplinary panel representing the Infectious Diseases Society of America (IDSA), the American Academy of Neurology (AAN), and the American College of Rheumatology (ACR). The scope of this guideline includes prevention of Lyme disease, and the diagnosis and treatment of Lyme disease presenting as erythema migrans, Lyme disease complicated by neurologic, cardiac, and rheumatologic manifestations, Eurasian manifestations of Lyme disease, and Lyme disease complicated by coinfection with other tick-borne pathogens. This guideline does not include comprehensive recommendations for babesiosis and tick-borne rickettsial infections, which are published in separate guidelines. The target audience for this guideline includes primary care physicians and specialists caring for this condition such as infectious diseases specialists, emergency physicians, internists, pediatricians, family physicians, neurologists, rheumatologists, cardiologists and dermatologists in North America.

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Meningioangiomatosis

Alexandre Roux,  View ORCID ProfileMarc Zanello, Rossella Letizia Mancusi, Megan E.H. Still,  View ORCID ProfileF?bio A. Nascimento, Arnault Tauziede-Espariat, Gilles Huberfeld, Gilles Zah-Bi, Edouard Dezamis, Jean-François Meder, Marie Bourgeois, Eduardo Parraga, Fabrice Chretien,  View ORCID ProfilePascale Varlet, Catherine Oppenheim, Emmanuèle Lechapt-Zalcman, Johan Pallud

doi : 10.1212/WNL.0000000000011372

February 09, 2021; 96 (6) 

Background Meningioangiomatosis is a poorly studied, rare, benign, and epileptogenic brain lesion.

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You Know I Love You, Right?

James Dolbow

doi : 10.1212/WNL.0000000000011225

February 09, 2021; 96 (6) 

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Bilateral High Signal Intensity on DWI of the Precentral Cortices in Mitochondriopathy Due to MTO1 Mutation and MT-TF Variant

Guillaume Dorcet,  View ORCID ProfileVincent Fabry,  View ORCID ProfileFabrice Bonneville,  View ORCID ProfilePascal Cintas

doi : 10.1212/WNL.0000000000011374

February 09, 2021; 96 (6) 

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Pearls & Oy-sters: Divergence Nystagmus

Eunjin Kwon, Ju-Young Lee, Hyo-Jung Kim, Jeong-Yoon Choi,  View ORCID ProfileJi-Soo Kim

doi : 10.1212/WNL.0000000000011273

February 09, 2021; 96 (6) 

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Opinion and Special Articles: Cerebellar Ataxia and Liver Failure Complicating IPEX Syndrome

Joshua Rim, Melissa Byler, Ariane Soldatos, Luigi Notarangelo, Emily Leibovitch, Steven Jacobson, Mark Gorman, Robert Roger Lebel, Klaus Werner

doi : 10.1212/WNL.0000000000011195

February 09, 2021; 96 (6) 

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Teaching NeuroImages: The Venous System and Developmental Venous Anomalies

Anthony S. Larson, Harry Cloft, Ivan D. Carabenciov, Giuseppe Lanzino

doi : 10.1212/WNL.0000000000010815

February 09, 2021; 96 (6) 

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Teaching Video NeuroImages: Wall Enhancement With Slow Blood Flow and Thrombosis Prior to Basilar Aneurysm Rupture

Fei Peng, Miaoqi Zhang, Hao Niu, Xin Feng, Xin Tong, Baorui Zhang, Fei Yuan, Chun Yuan, Rui Li, Aihua Liu

doi : 10.1212/WNL.0000000000010820

February 09, 2021; 96 (6) 

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Editors' Note: Evaluation of Efficacy and Tolerability of First-Line Therapies in NMOSD

Aravind Ganesh, Steven Galetta

doi : 10.1212/WNL.0000000000011400

February 09, 2021; 96 (6) 

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Reader Response: Evaluation of Efficacy and Tolerability of First-Line Therapies in NMOSD

M. Isabel Leite

doi : 10.1212/WNL.0000000000011401

February 09, 2021; 96 (6) 

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Author Response: Evaluation of Efficacy and Tolerability of First-Line Therapies in NMOSD

 View ORCID ProfileHelene Zephir, Julien Poupart, Romain Deschamps,  View ORCID ProfileBertrand Audoin, Jonathan Ciron,  View ORCID ProfileElisabeth Maillart, Caroline Papeix,  View ORCID ProfileNicolas Collongues, Bertrand Bourre, Mickaël Cohen, Sandrine Wiertlewski, Olivier Outteryck, David Laplaud, Sandra Vukusic, Romain Marignier,  View ORCID ProfileJonathan Giovannelli

doi : 10.1212/WNL.0000000000011403

February 09, 2021; 96 (6) 

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Clinical Practice Guidelines by the Infectious Diseases Society of America, American Academy of Neurology, and American College of Rheumatology

doi : 10.1212/WNL.0000000000011422

February 09, 2021; 96 (6) 

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