Neurology




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The Role of Hereditary Hemorrhagic Telangiectasia in Neurologic Practice.

Taylor, John Michael MD; Hammill, Adrienne M. MD, PhD

doi : 10.1212/WNL.0000000000207384

Volume 100(23) pgs. 1077-1120,e2324-e2423 June 6, 2023

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The Importance of Understanding Neighborhood Environments in Neurology Care.

Kobau, Rosemarie MPH; Moura, Lidia M. V. R. MD, MPH

doi : 10.1212/WNL.0000000000207390

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CSF in Guillain-Barre Syndrome: It's a Matter of Timing.

Sejvar, James MD

doi : 10.1212/WNL.0000000000207203

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Brain Abscess and Stroke in Children and Adults With Hereditary Hemorrhagic Telangiectasia: Analysis of a Large National Claims Database.

White, Andrew J. MD; Marmor, Itay MD; Peacock, Kate M.; Nickel, Katelin B.; Zavadil, Jessica MD, PhD; Olsen, Margaret A. PhD, MPH

doi : 10.1212/WNL.0000000000207269

Hereditary hemorrhagic telangiectasia (HHT) is an inherited disease associated with pathogenic variants in transforming growth factor-[beta] signaling pathway-related genes, resulting in abnormal vascular development in various organs.

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Short-term Trajectories of Poststroke Cognitive Function: A STROKOG Collaboration Study.

Lo, Jessica W. MSc; Crawford, John D. PhD; Desmond, David W. PhD; Bae, Hee-Joon MD; Lim, Jae-Sung MD, PhD; Godefroy, Olivier MD, PhD; Roussel, Martine PhD; Kohler, Sebastian PhD; Staals, Julie MD, PhD; Verhey, Frans MD, PhD; Chen, Christopher MD; Xu, Xin PhD; Chong, Eddie J. BA; Kandiah, Nagaendran FRCP, MD; Bordet, Regis MD; Dondaine, Thibaut PhD; Mendyk, Anne-Marie RN; Brodaty, Henry MD; Traykov, Latchezar MD; Mehrabian, Shima MD; Petrova, Neli MD; Lipnicki, Darren M. PhD; Lam, Ben Chun Pan PhD; Sachdev, Perminder S. MD, PhD, FRANZCP; for the Stroke and Cognition (STROKOG) Collaboration; for the Stroke and Cognition (STROKOG) Collaboration; Akinyemi, Rufus PhD; Bath, Philip FRCP; Brodtmann, Amy PhD; Douiri, Abdel PhD; Dichgans, Martin MD; Hoffmann, Michael MD; Kalaria, Raj FRCPath; Jokinen, Hanna PhD; de Kort, Paul MD, PhD; Lau, Gary FRCP; Lee, Byung-Chul MD; Linden, Thomas MD; Markus, Hugh DM, FMed Sci; Mok, Vincent MD, FRCP; Mrowiec, Aleksandra Klimkowicz- MD, PhD; O'Sullivan, Michael PhD; Sabayan, Behnam MD; Srikanth, Velandai PhD; Wardlaw, Joanna MD; Cordonnier, Charlotte MD; Xu, Qun MD, PhD; Yu, Kyung-Ho MD, PhD

doi : 10.1212/WNL.0000000000207281

Past studies on poststroke cognitive function have focused on the average performance or change over time, but few have investigated patterns of cognitive trajectories after stroke.

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Association of Handgrip Strength Weakness and Asymmetry With Incidence of Motoric Cognitive Risk Syndrome in the China Health and Retirement Longitudinal Study.

Jia, Shuli MD *; Zhao, Wanyu PhD *; Ge, Meiling PhD; Zhou, Lixing PhD; Sun, Xuelian MD; Zhao, Yunli MD; Dong, Birong MD

doi : 10.1212/WNL.0000000000207308

Motoric cognitive risk (MCR) syndrome is a type of pre-dementia. It is defined as the co-occurrence of subjective cognitive complaints and a slow gait speed.

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Association of Neighborhood Deprivation With Cognitive and Mood Outcomes in Adults With Pharmacoresistant Temporal Lobe Epilepsy.

Busch, Robyn M. PhD *; Dalton, Jarrod E. PhD *; Jehi, Lara MD; Ferguson, Lisa MA; Krieger, Nikolas I. MS; Struck, Aaron F. MD; Hermann, Bruce P. PhD

doi : 10.1212/WNL.0000000000207266

Temporal lobe epilepsy (TLE) is the most common adult form of epilepsy and is associated with a high risk of cognitive deficits and depressed mood.

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Prospective Multicenter Validation of a Simple Blood Test for the Diagnosis of Glut1 Deficiency Syndrome.

Mochel, Fanny MD, PhD; Gras, Domitille MD; Luton, Marie-Pierre *; Nizou, Manon *; Giovannini, Donatella *; Delattre, Caroline; Aubart, Melodie MD, PhD; Barth, Magalie MD; De Saint-Martin, Anne MD; Doummar, Diane MD; Essid, Nouha MD; Garros, Alexa MD; Le Camus, Caroline Hachon MD; Hoebeke, Celia MD; The Tich, Sylvie Nguyen MD, PhD; Perivier, Maximilien MD; Rivera, Serge MD; Rolland, Anne MD; Roubertie, Agathe MD, PhD; Sarret, Catherine MD, PhD; Sevin, Caroline MD; Ville, Dorothee MD; Sitbon, Marc PhD; Costa, Jean-Marc PharmD; Pons, Roser MD, PhD; Garcia-Cazorla, Angels MD, PhD; Vuillaumier, Sandrine PhD; Petit, Vincent PhD; Boespflug-Tanguy, Odile MD, PhD; De Vivo, Darryl C. MD; for the MetaGlut1 Study Group; for the MetaGlut1 Study Group; Mochel, Fanny MD, PhD; Gras, Domitille MD; Luton, Marie-Pierre PhD; Nizou, Manon MS; Giovannini, Donatella PhD; Delattre, Caroline MS; Aubart, Melodie MD, PhD; Barth, Magalie MD; De Saint- Martin, Anne MD; Doummar, Diane MD; Essid, Nouha MD; Garros, Alexa MD; Hachon-Le Camus, Caroline MD; Hoebeke, Celia MD; The Tich, Sylvie Nguyen MD, PhD; Perivier, Maximilien MD; Rivera, Serge MD; Rolland, Anne MD; Roubertie, Agathe MD, PhD; Sarret, Catherine MD, PhD; Sevin, Caroline MD, PhD; Ville, Dorothee MD; Sitbon, Marc PhD; Costa, Jean-Marc PharmD; Pons, Roser MD, PhD; Cazorla, Angeles Garcia- MD, PhD; Vuillaumier-Barrot, Sandrine PhD; Petit, Vincent PhD; Tanguy, Odile Boespflug- MD, PhD; De Vivo, Darryl C MD

doi : 10.1212/WNL.0000000000207296

GLUT1 deficiency syndrome (Glut1DS) is a treatable neurometabolic disease that causes a wide range of neurologic symptoms in children and adults.

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Analyses of Head-Impulse Tests in Patients With Posterior Circulation Stroke and Vestibular Neuritis.

Kim, Sung-Hwan MD; Lee, Sun-Uk MD, PhD; Cho, Bang-Hoon MD; Cho, Kyung-Hee MD, PhD; Yu, Sungwook MD, PhD; Kim, Byung-Jo MD, PhD; Kim, Ji-Soo MD, PhD

doi : 10.1212/WNL.0000000000207299

The interpretation of video head-impulse tests (video-HITs) can often be complicated, limiting their clinical utility in acute vestibular syndrome.

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Development and Evaluation of a Simulation-Based Algorithm to Optimize the Planning of Interim Analyses for Clinical Trials in ALS.

van Unnik, Jordi W.J. MSc; Nikolakopoulos, Stavros PhD; Eijkemans, Marinus J.C. PhD; Gonzalez-Bermejo, Jesus MD, PhD; Bruneteau, Gaelle MD, PhD; Morelot-Panzini, Capucine MD, PhD; van den Berg, Leonard H. MD, PhD; Cudkowicz, Merit E. MD; McDermott, Christopher J. MD, PhD; Similowski, Thomas MD, PhD; van Eijk, Ruben P.A. MD, PhD; for the DiPALS, RespiStimALS and Ceftriaxone Study Group

doi : 10.1212/WNL.0000000000207306

Late-phase clinical trials for neurodegenerative diseases have a low probability of success. In this study, we introduce an algorithm that optimizes the planning of interim analyses for clinical trials in amyotrophic lateral sclerosis (ALS) to better use the time and resources available and minimize the exposure of patients to ineffective or harmful drugs.

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An Introduction to Individual Participant Data Meta-analysis.

Veroniki, Areti Angeliki PhD; Seitidis, Georgios MSc; Tsivgoulis, Georgios MD, PhD; Katsanos, Aristeidis H. MD; Mavridis, Dimitris PhD

doi : 10.1212/WNL.0000000000207078

AB Meta-analysis using individual participant data (IPD-MA) from randomized controlled trials (RCTs) can strengthen evidence used for decision making and is considered the "gold standard" approach. In this study, we present the importance, properties, and main approaches of conducting an IPD-MA.

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Clinical Reasoning: Drug Resistant Epilepsy in a 61-Year-Old Man With Abnormal MRI Brain Findings and Management With Vagal Nerve Stimulator.

Mankad, Jigar Prakashchandra MD; Lavingia, Janki Kiran MD

doi : 10.1212/WNL.0000000000206890

AB A 66-year-old man with seizures that started at 61 years eventually developed drug-resistant epilepsy and was managed with medications and vagal nerve stimulation.

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Teaching NeuroImage: Imaging and Pathologic Findings in SARS-CoV-2-Related Acute Demyelinating Encephalomyelitis.

Lalla, Rakhee DO; Narasimhan, Ramya MD; Abdalkader, Mohamad MD; Virmani, Deepti MD; Suchdev, Kushak MD; Moore, Brian MD, MEd; Cervantes-Arslanian, Anna MD

doi : 10.1212/WNL.0000000000207095

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Editors' Note: Neurology Education in 2035: The Neurology Future Forecasting Series.

Siegler, James E. MD; Galetta, Steven MD

doi : 10.1212/WNL.0000000000207440

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Reader Response: Neurology Education in 2035: The Neurology Future Forecasting Series.

Das, Saurav

doi : 10.1212/WNL.0000000000207441

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Author Response: Neurology Education in 2035: The Neurology Future Forecasting Series.

Moeller, Jeremy J.; Salas, Rachel M.E.

doi : 10.1212/WNL.0000000000207442

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Teaching NeuroImages: Optic Nerve Sheath Meningioma Presenting as Gaze-Evoked Amaurosis.

doi : 10.1212/WNL.0000000000201643

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CSF Findings in Relation to Clinical Characteristics, Subtype, and Disease Course in Patients With Guillain-Barre Syndrome.

Al-Hakem, Helle MD; Doets, Alex Y. MD, PhD; Stino, Amro Maher MD; Zivkovic, Sasha A. MD, PhD; Andersen, Henning MD, DMSc; Willison, Hugh J. MD, PhD; Cornblath, David R. MD; Gorson, Kenneth C. MD; Islam, Zhahirul PhD; Mohammad, Quazi Deen MD; Sindrup, Soren Hein MD, DMSc; Kusunoki, Susumu MD, PhD; Davidson,

doi : 10.1212/WNL.0000000000207282

To investigate CSF findings in relation to clinical and electrodiagnostic subtypes, severity, and outcome of Guillain-Barre syndrome (GBS) based on 1,500 patients in the International GBS Outcome Study

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Changes in Brain Activation Patterns During Working Memory Tasks in People With Post-COVID Condition and Persistent Neuropsychiatric Symptoms.

Chang, Linda MD, MS; Ryan, Meghann C. MS; Liang, Huajun MBBS, PhD; Zhang, Xin MS; Cunningham, Eric BS; Wang, Justin; Wilson, Eleanor MD, MHS; Herskovits, Edward H. MD, PhD; Kottilil, Shyamasundaran MBBS, PhD; Ernst, Thomas M. PhD

doi : 10.1212/WNL.0000000000207309

Post-COVID condition (PCC) is common and often involves neuropsychiatric symptoms. This study aimed to use blood oxygenation level-dependent fMRI (BOLD-fMRI) to assess whether participants with PCC had abnormal brain activation during working memory (WM) and whether the abnormal brain activation could predict cognitive performance, motor function, or psychiatric symptoms.

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