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Spotlight on the May 4 Issue
José G. Merino
doi : 10.1212/WNL.0000000000011864
May 04, 2021; 96 (18)
SLC32A1 One More Gene Contributing to the Solution of the Genetic Generalized Epilepsies Mystery
Renzo Guerrini, Carla Marini
doi : 10.1212/WNL.0000000000011854
May 04, 2021; 96 (18)
A New Way to Identify Promising Therapies for Progressive Multiple Sclerosis
Robert J. Fox, Richard J. Kryscio
doi : 10.1212/WNL.0000000000011862
May 04, 2021; 96 (18)
Embracing the Unknown in the Diagnosis of Traumatic Encephalopathy Syndrome
Lili-Naz Hazrati, Nicole Schwab
doi : 10.1212/WNL.0000000000011847
May 04, 2021; 96 (18)
Association of SLC32A1 Missense Variants With Genetic Epilepsy With Febrile Seizures Plus
Sarah E. Heron, Brigid M. Regan, Rebekah V. Harris, Alison E. Gardner,Matthew J. Coleman, View ORCID ProfileMark F. Bennett, Bronwyn E. Grinton,Katherine L. Helbig, Michael R. Sperling, Sheryl Haut, Eric B. Geller, Peter Widdess-Walsh, James T. Pelekanos,Melanie Bahlo, Slavé Petrovski, Erin L. Heinzen, Michael S. Hildebrand, Mark A. Corbett, Ingrid E. Scheffer,Jozef Gécz,Samuel F. Berkovic
doi : 10.1212/WNL.0000000000011855
May 04, 2021; 96 (18)
Objective To identify the causative gene in a large unsolved family with genetic epilepsy with febrile seizures plus (GEFS+), we sequenced the genomes of family members, and then determined the contribution of the identified gene to the pathogenicity of epilepsies by examining sequencing data from 2,772 additional patients.
Network-Related Changes in Neurotransmitters and Seizure Propagation During Rodent Epileptogenesis
Roni Dhaher, Shaun E. Gruenbaum,Mani Ratnesh S. Sandhu,Sigrid Ottestad-Hansen, View ORCID ProfileNathan Tu, Yue Wang,Tih-Shih W. Lee,Ketaki Deshpande,Dennis D. Spencer, Niels Christian Danbolt,Hitten P. Zaveri,Tore Eid
doi : 10.1212/WNL.0000000000011846
May 04, 2021; 96 (18)
Objective To test the hypothesis that glutamate and GABA are linked to the formation of epilepsy networks and the triggering of spontaneous seizures, we examined seizure initiation/propagation characteristics and neurotransmitter levels during epileptogenesis in a translationally relevant rodent model of mesial temporal lobe epilepsy.
Age-at-Onset and APOE-Related Heterogeneity in Pathologically Confirmed Sporadic Alzheimer Disease
Denis S. Smirnov, Douglas Galasko, Annie Hiniker, Steven D. Edland, David P. Salmon
doi : 10.1212/WNL.0000000000011772
May 04, 2021; 96 (18)
Objective To characterize age-related clinical heterogeneity in Alzheimer disease (AD) and determine whether it is modified by APOE genotype or concomitant non-AD pathology, we analyzed data from 1,750 patients with sporadic, pathologically confirmed severe AD.
Dual Sensory Impairment and Cognitive Impairment in the Korean Longitudinal Elderly Cohort
Gihwan Byeon, Gyu Han Oh,Jin Hyeong Jhoo,Jae-Won Jang,Jong Bin Bae,Ji Won Han,Tae Hui Kim,Kyung Phil Kwak,Bong Jo Kim,Shin Gyeom Kim, Jeong Lan Kim,Seok Woo Moon,Joon Hyuk Park,Seung-Ho Ryu, Jong Chul Youn, Dong Woo Lee,Seok Bum Lee, Jung Jae Lee,Dong Young Lee,Ki Woong Kim
doi : 10.1212/WNL.0000000000011845
May 04, 2021; 96 (18)
Objective To investigate the effects of single sensory impairment (SSI; visual or auditory) or dual sensory impairment (DSI; visual and auditory) on dementia and longitudinal changes of neuropsychological test scores.
Plasma Neurofilament Light for Prediction of Disease Progression in Familial Frontotemporal Lobar Degeneration
Julio C. Rojas, Ping Wang, Adam M. Staffaroni, Carolin Heller,Yann Cobigo, Amy Wolf, Sheng-Yang M. Goh, Peter A. Ljubenkov, Hilary W. Heuer,Jamie C. Fong, Joanne B. Taylor, Eliseo Veras, Linan Song, Andreas Jeromin, David Hanlon, Lili Yu, Arvind Khinikar, Rajeev Sivasankaran, Agnieszka Kieloch, Marie-Anne Valentin, Anna M. Karydas, Laura L. Mitic, Rodney Pearlman, John Kornak, Joel H. Kramer, Bruce L. Miller, Kejal Kantarci, David S. Knopman, Neill Graff-Radford, Leonard Petrucelli, Rosa Rademakers, David J. Irwin, Murray Grossman, Eliana Marisa Ramos,Giovanni Coppola, Mario F. Mendez, Yvette Bordelon,Bradford C. Dickerson,Nupur Ghoshal, Edward D. Huey, Ian R. Mackenzie, Brian S. Appleby, Kimiko Domoto-Reilly,Ging-Yuek R. Hsiung, Arthur W. Toga, Sandra Weintraub, Daniel I. Kaufer, Diana Kerwin, Irene Litvan,Chiadikaobi U. Onyike,Alexander Pantelyat,Erik D. Roberson, Maria C. Tartaglia, Tatiana Foroud, Weiping Chen, Julie Czerkowicz, Danielle L. Graham, John C. van Swieten, Barbara Borroni, Raquel Sanchez-Valle, Fermin Moreno,Robert Laforce, Caroline Graff, Matthis Synofzik,Daniela Galimberti, James B. Rowe, Mario Masellis, Elizabeth Finger,Rik Vandenberghe, Alexandre de Mendonça, Fabrizio Tagliavini, Isabel Santana, Simon Ducharme, Chris R. Butler, Alexander Gerhard, Johannes Levin,Adrian Danek,Markus Otto, Sandro Sorbi,David M. Cash, Rhian S. Convery, Martina Bocchetta, Martha Foiani, Caroline V. Greaves,Georgia Peakman, Lucy Russell, Imogen Swift,Emily Todd, Jonathan D. Rohrer, Bradley F. Boeve, Howard J. Rosen, Adam L. Boxer, on behalf of the ALLFTD and GENFI consortia
doi : 10.1212/WNL.0000000000011848
May 04, 2021; 96 (18)
Objective We tested the hypothesis that plasma neurofilament light chain (NfL) identifies asymptomatic carriers of familial frontotemporal lobar degeneration (FTLD)–causing mutations at risk of disease progression.
Repurposing Domperidone in Secondary Progressive Multiple Sclerosis
Marcus W. Koch, Kayla Sage, Sharanjit Kaur, Janet Kim, Graziela Cerchiaro, V. Wee Yong, Gary R. Cutter, Luanne M. Metz
doi : 10.1212/WNL.0000000000011863
May 04, 2021; 96 (18)
Objective To assess whether treatment with the generic drug domperidone can reduce the progression of disability in secondary progressive multiple sclerosis (SPMS), we conducted a phase 2 futility trial following the Simon 2-stage design.
Randomized Study of Metoclopramide Plus Diphenhydramine for Acute Posttraumatic Headache
Benjamin W. Friedman, Eddie Irizarry, Darnell Cain, Arianna Caradonna, Mia T. Minen, Clemencia Solorzano, Eleftheria Zias, David Zybert, Michael McGregor, Polly E. Bijur, E. John Gallagher
doi : 10.1212/WNL.0000000000011822
May 04, 2021; 96 (18)
Objective To determine whether IV metoclopramide 20 mg + diphenhydramine 25 mg (M + D) was more efficacious than IV placebo for acute moderate or severe posttraumatic headache in the emergency room.
Development of a Sensitive Diagnostic Assay for Parkinson Disease Quantifying ?-Synuclein–Containing Extracellular Vesicles
Zhen Hong, Chen Tian, Tessandra Stewart, Patrick Aro, David Soltys, Matt Bercow, Lifu Sheng, Kayla Borden, Tarek Khrisat, Catherine Pan,Cyrus P. Zabetian, Elaine R. Peskind, Joseph F. Quinn, Thomas J. Montine, Jan Aasly,Min Shi, Jing Zhang
doi : 10.1212/WNL.0000000000011853
May 04, 2021; 96 (18)
Objective To develop a reliable and fast assay to quantify the ?-synuclein (?-syn)–containing extracellular vesicles (EVs) in CSF and to assess their diagnostic potential for Parkinson disease (PD).
What's Happening in Neurology® Clinical Practice
doi : 10.1212/WNL.0000000000011861
May 04, 2021; 96 (18)
What's Happening in Neurology® Neuroimmunology & Neuroinflammation
doi : 10.1212/WNL.0000000000011859
May 04, 2021; 96 (18)
National Institute of Neurological Disorders and Stroke Consensus Diagnostic Criteria for Traumatic Encephalopathy Syndrome
Douglas I. Katz, Charles Bernick, David W. Dodick, Jesse Mez, Megan L. Mariani, Charles H. Adler, Michael L. Alosco, Laura J. Balcer, Sarah J. Banks, William B. Barr, David L. Brody, Robert C. Cantu, Kristen Dams-O'Connor, Yonas E. Geda, Barry D. Jordan, Thomas W. McAllister, Elaine R. Peskind, Ronald C. Petersen, Jennifer V. Wethe, Ross D. Zafonte, ?imear M. Foley, Debra J. Babcock, Walter J. Koroshetz, Yorghos Tripodis, Ann C. McKee, Martha E. Shenton, Jeffrey L. Cummings,Eric M. Reiman, Robert A. Stern
doi : 10.1212/WNL.0000000000011850
May 04, 2021; 96 (18)
Objective To develop evidence-informed, expert consensus research diagnostic criteria for traumatic encephalopathy syndrome (TES), the clinical disorder associated with neuropathologically diagnosed chronic traumatic encephalopathy (CTE).
Pearls & Oy-sters: Eyes-Open Coma
Daniel Kondziella,Jennifer A. Frontera
doi : 10.1212/WNL.0000000000011715
May 04, 2021; 96 (18)
Clinical Reasoning: A Teenager With Shortness of Breath and Difficulty Walking
Suzanne C. Liu, Jason T. Poon, Meghan S. Candee
doi : 10.1212/WNL.0000000000011544
May 04, 2021; 96 (18)
Teaching NeuroImages: Magnetic Susceptibility–Weighted Imaging Mapped the Migratory Route of a Paragonimus in Brain
Ke Yu, Mingjie Zhang
doi : 10.1212/WNL.0000000000011492
May 04, 2021; 96 (18)
Teaching Video NeuroImages: Almost No Eye Movements to the Left
Michael Strupp, Claudia Frenzel, Jun Thorsteinsdottir
doi : 10.1212/WNL.0000000000011234
May 04, 2021; 96 (18)
Editors' Note: Education Research: The Medical Student Perspective on Challenging Conversations
James E. Siegler, Steven Galetta
doi : 10.1212/WNL.0000000000011860
May 04, 2021; 96 (18)
Reader Response: Education Research: The Medical Student Perspective on Challenging Conversations
Syed Amin, Raena Ullah
doi : 10.1212/WNL.0000000000011866
May 04, 2021; 96 (18)
Author Response: Education Research: The Medical Student Perspective on Challenging Conversations
Rheaya WillisRoy E. Strowd,Mary C. Barks, Rachel E. Salas, Charlene E. Gamaldo, Monica E. Lemmon
doi : 10.1212/WNL.0000000000011865
May 04, 2021; 96 (18)
Editors' Note: Effect of Ocrelizumab on Vaccine Responses in Patients With Multiple Sclerosis: The VELOCE Study
James E. Siegler, Steven Galetta
doi : 10.1212/WNL.0000000000011869
May 04, 2021; 96 (18)
Reader Response: Effect of Ocrelizumab on Vaccine Responses in Patients With Multiple Sclerosis: The VELOCE Study
Nicole Hahn
doi : 10.1212/WNL.0000000000011867
May 04, 2021; 96 (18)
Author Response: Effect of Ocrelizumab on Vaccine Responses in Patients With Multiple Sclerosis: The VELOCE Study
Amit Bar-Or, Ann Herman, Daniela Stokmaier
doi : 10.1212/WNL.0000000000011868
May 04, 2021; 96 (18)
