Neurology

Moore, Elizabeth E. PhD; Liu, Dandan PhD; Li, Judy BS; Schimmel, Samantha J.; Cambronero, Francis E. AB; Terry, James G. MS; Nair, Sangeeta DVM, MS; Pechman, Kimberly R. PhD; Moore, Marissa E. MS; Bell, Susan P. MBBS, MSCI; Beckman, Joshua A. MD, MS; Gifford, Katherine A. PsyD; Hohman, Timothy J. PhD; Blennow, Kaj MD, PhD; Zetterberg, Henrik MD, PhD; Carr, John Jeffrey MD, MSc; Jefferson, Angela L. PhD

doi : 10.1212/WNL.0000000000012257

Volume 97(4), 27 July 2021, p e329-e340

To test the hypothesis that increased aortic stiffening is associated with greater CSF evidence of core Alzheimer disease pathology ([beta]-amyloid [A[beta]], phosphorylated tau [p-tau]), neurodegeneration (total tau [t-tau]), synaptic dysfunction (neurogranin), neuroaxonal injury (neurofilament light [NFL]), and neuroinflammation (YKL-40, soluble triggering receptor expressed on myeloid cells 2 [sTREM2]), we analyzed pulse wave velocity (PWV) data and CSF data among older adults.

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Association of Serum Magnesium Levels With Risk of Intracranial Aneurysm: A Mendelian Randomization Study

Larsson, Susanna C. PhD; Gill, Dipender BMBCh, PhD

doi : 10.1212/WNL.0000000000012244

Volume 97(4), 27 July 2021, p e341-e344

Magnesium has been implicated in regulating blood pressure and vascular endothelial cell function, but its role in the pathophysiology of intracranial aneurysm is not known. Here we performed a Mendelian randomization analysis to investigate the association between serum magnesium concentration and risk of intracranial aneurysm.

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Association of SUMOylation Pathway Genes With Stroke in a Genome-Wide Association Study in India

Kumar, Amit PhD*; Chauhan, Ganesh PhD*; Sharma, Shriram DM; Dabla, Surekha DM; Sylaja, P.N. DM; Chaudhary, Neera DM; Gupta, Salil DM; Agrawal, Chandra Sekhar DM; Anand, Kuljeet Singh DM; Srivastava, Achal Kumar DM; Vibha, Deepti DM; Sagar, Ram PhD; Raj, Ritesh MSc; Maheshwari, Ankita MSc; Vivekanandhan, Subbiah PhD; Kaul, Bhavna DM; Raghavan, Samudrala DM; Gorthi, Sankar Prasad DM; Mohania, Dheeraj PhD; Kaushik, Samander PhD; Yadav, Rohtas Kanwar MD; Hazarika, Anjali MBBS; Sharma, Pankaj DM, PhD; Prasad, Kameshwar DM

doi : 10.1212/WNL.0000000000012258

Volume 97(4), 27 July 2021, p e345-e356

To undertake a genome-wide association study (GWAS) to identify genetic variants for stroke in an Indian population.

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Impact of the Surgical Approach to Thymectomy Upon Complete Stable Remission Rates in Myasthenia Gravis: A Meta-analysis

Solis-Pazmino, Paola MD*; Baiu, Ioana MD, MPH*; Lincango-Naranjo, Eddy MD; Trope, Winston; Prokop, Larry; Ponce, Oscar J. MD; Shrager, Joseph B. MD

doi : 10.1212/WNL.0000000000012153

Volume 97(4), 27 July 2021, p e357-e368

To determine whether the available operative techniques for thymectomy in myasthenia gravis (MG) confer variable chances for achieving complete stable remission (CSR), we performed a meta-analysis of comparative studies of surgical approaches to thymectomy.

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Accuracy of a Deep Learning System for Classification of Papilledema Severity on Ocular Fundus Photographs

Vasseneix, Caroline MD; Najjar, Raymond P. PhD; Xu, Xinxing PhD; Tang, Zhiqun PhD; Loo, Jing Liang MBBS, MMed, FRCS(Ed); Singhal, Shweta MBBS; Tow, Sharon MBBS; Milea, Leonard; Ting, Daniel Shu Wei MD, PhD; Liu, Yong PhD; Wong, Tien Y. MD, PhD; Newman, Nancy J. MD; Biousse, Valerie MD; Milea, Dan MD, PhD; on behalf of the BONSAI Group; on behalf of the BONSAI Group; Gohier, Philippe MD; Miller, Neil MD; Padungkiatsagul, Tanyatuth MD; Poonyathalang, Anuchit MD; Suwan, Yanin MD; Vanikieti, Kavin MD; Amore, Giulia MD; Barboni, Piero MD; Carbonelli, Michele MD; Carelli, Valerio MD, PhD; La Morgia, Chiara MD, PhD; Romagnoli, Martina PhD; Rougier, Marie-Benedicte MD, PhD; Ambika, Selvakumar DO, DNB; Swetha, Komma DO, DNB; Fonseca, Pedro MD; Raimundo, Miguel MD; Hamann, Steffen MD, PhD, FEBO; Karlesand, Isabelle MD; Fuhrmann, Lars MD; Kuchlin, Sebastian MD; Lagreze, Wolf Alexander MD; Sanda, Nicolae MD, PhD; Thumann, Gabriele MD, PD; Aptel, Florent MD, PhD; Chiquet, Christophe MD, PhD; Liu, Kaiqun MD; Yang, Hui MD, PhD; Chan, Carmen KM MRCP, FRCSEd(Ophth); Chan, Noel CY FRCSEd(Ophth); Cheung, Carol Y PhD; Ha Chau, Tran Thi MD; Acheson, James BM, MRCP (UK) , FRCOphth; Habib, Maged S MD, MSc Ophth, FRCS, FRCOphth ; Jurkute, Neringa MD, FEBO ; Yu-Wai-Man, Patrick PhD, MB, BS, FRCPath, FRCOphth ; Kho, Richard MD; Jonas, Jost B MD; Chen, John J. MD, PhD; Sabbagh, Nouran MD; VignalClermont, Catherine MD; Hage, Rabih MD; Khanna, Raoul Kanav MD; Hwang, Jeong-Min MD; Kim, Dong Hyun MD; Yang, Hee Kyung MD; Aung, Tin MD, PhD; Cheng, Ching-Yu MD, PhD; Lamoureux, Ecosse MSc, PhD; Schmetterer, Leopold PhD; Jiang, Zhubo MSc; Fraser, Clare L MBBS, MMed, FRANZCO ; Mejico, Luis J. MD; Fard, Masoud Aghsaei MD

doi : 10.1212/WNL.0000000000012226

Volume 97(4), 27 July 2021, p e369-e377

To evaluate the performance of a deep learning system (DLS) in classifying the severity of papilledema associated with increased intracranial pressure on standard retinal fundus photographs.

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Parkinson Disease-Related Brain Metabolic Patterns and Neurodegeneration in Isolated REM Sleep Behavior Disorder

Shin, Jung Hwan MD, PhD; Lee, Jee-Young MD, PhD; Kim, Yu-Kyeong MD, PhD; Yoon, Eun Jin PhD; Kim, Heejung PhD; Nam, Hyunwoo MD, PhD; Jeon, Beomseok MD, PhD

doi : 10.1212/WNL.0000000000012228

Volume 97(4), 27 July 2021, p e378-e388

To elucidate the role of Parkinson disease (PD)-related brain metabolic patterns as a biomarker in isolated REM sleep behavior disorder (iRBD) for future disease conversion.

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Contralateral Sensory and Pain Perception Changes in Patients With Unilateral Neuropathy

Enax-Krumova, Elena MD; Attal, Nadine MD, PhD; Bouhassira, Didier MD, PhD; Freynhagen, Rainer MD; Gierthmuhlen, Janne MD; Hansson, Per MD, DMSci, DDS; Kuehler, Bianca M. MD; Maier, Christoph MD; Sachau, Juliane MD; Segerdahl, Marta MD, PhD; Tolle, Thomas MD; Treede, Rolf-Detlef MD; Ventzel, Lise MD, PhD; Baron, Ralf MD; Vollert, Jan PhD

doi : 10.1212/WNL.0000000000012229

Volume 97(4), 27 July 2021, p e389-e402

To test whether contralateral sensory abnormalities in the clinically unaffected area of patients with unilateral neuropathic pain are due to the neuropathy or pain mechanisms.

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Effects of Age and Disease Duration on Excess Mortality in Patients With Multiple Sclerosis From a French Nationwide Cohort

Rollot, Fabien MSc; Fauvernier, Mathieu PhD; Uhry, Zoe MSc; Vukusic, Sandra PhD; Bossard, Nadine PhD; Remontet, Laurent MSc; Leray, Emmanuelle PhD; on behalf of the OFSEP Investigators; on behalf of the OFSEP Investigators; Brochet, Bruno MD, PhD; Casey, Romain PhD; Cotton, Francois MD, PhD; De Seze, Jerome MD, PhD; Mansuy, Lucilla; Douek, Pascal MD; Guillemin, Francis MD, PhD; Laplaud, David MD, PhD; LebrunFrenay, Christine MD, PhD; Moreau, Thibault MD, PhD; Olaiz, Javier PhD; Pelletier, Jean MD, PhD; Bully, Claire Rigaud; Stankoff, Bruno MD, PhD; Vukusic, Sandra MD, PhD; Marignier, Romain MD, PhD; Debouverie, Marc MD, PhD; Edan, Gilles MD, PhD; Ciron, Jonathan MD; Ruet, Aurelie MD, PhD; Collongues, Nicolas MD, PhD; Vermersch, Patrick MD, PhD; Cohen, Mikael MD; Fromont, Agnes MD, PhD; Defer, Gilles MD; Wiertlewski, Sandrine MD; Clavelou, Pierre MD, PhD; Labauge, Pierre MD, PhD; Berger, Eric MD; Audoin, Bertrand MD, PhD; Heinzlef, Olivier MD; Thouvenot, Eric MD, PhD; Camdessanche, Jean Philippe MD, PhD

doi : 10.1212/WNL.0000000000012224

Volume 97(4), 27 July 2021, p e403-e413

To determine the effects of current age and disease duration on excess mortality in multiple sclerosis (MS), we describe the dynamics of excess death rates over these 2 time scales and study the effect of age at MS clinical onset on these dynamics, separately in each initial phenotype.

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Clinical and Radiologic Features, Pathology, and Treatment of Balo Concentric Sclerosis

Jolliffe, Evan A. MBChB; Guo, Yong MD, PhD; Hardy, Todd A. MBBS; Morris, P. Pearse MBBCh; Flanagan, Eoin P. MBBCh; Lucchinetti, Claudia F. MD; Tobin, W. Oliver MBBCh, BAO, PhD

doi : 10.1212/WNL.0000000000012230

Volume 97(4), 27 July 2021, p e414-e422

To describe clinical, radiologic, and pathologic features of Balo concentric sclerosis (BCS) and assess overlap between BCS and other CNS inflammatory demyelinating diseases.

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Can Transcranial Direct Current Stimulation Enhance Poststroke Motor Recovery?: Development of a Theoretical Patient-Tailored Model

Hordacre, Brenton PhD; McCambridge, Alana B. PhD; Ridding, Michael C. PhD; Bradnam, Lynley V. PhD

doi : 10.1212/WNL.0000000000012187

Volume 97(4), 27 July 2021, p 170-180

New treatments that can facilitate neural repair and reduce persistent impairments have significant value in promoting recovery following stroke. One technique that has gained interest is transcranial direct current stimulation (tDCS) as early research suggested it could enhance plasticity and enable greater behavioral recovery. However, several studies have now identified substantial intersubject variability in response to tDCS and clinical trials revealed insufficient evidence of treatment effectiveness. A possible explanation for the varied and negative findings is that the physiologic model of stroke recovery that researchers have used to guide the application of tDCS-based treatments in stroke is overly simplistic and does not account for stroke heterogeneity or known determinants that affect the tDCS response. Here, we propose that tDCS could have a more clearly beneficial role in enhancing stroke recovery if greater consideration is given to individualizing treatment. By critically reviewing the proposed mechanisms of tDCS, stroke physiology across the recovery continuum, and known determinants of tDCS response, we propose a new, theoretical, patient-tailored approach to delivering tDCS after stroke. The proposed model includes a step-by-step principled selection strategy for identifying optimal neuromodulation targets and outlines key areas for further investigation. Tailoring tDCS treatment to individual neuroanatomy and physiology is likely our best chance at producing robust and meaningful clinical benefit for people with stroke and would therefore accelerate opportunities for clinical translation.

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Clarence J. Gibbs Effect and the "Creutzfeldt-Jakob Disease" Eponym

Lanska, Douglas J. MD, MS, MSPH

doi : 10.1212/WNL.0000000000012199

Volume 97(4), 27 July 2021, p 181-187

In 2014, American neurologist and Nobel laureate Stanley Prusiner reported that microbiologist Clarence Joseph Gibbs at the US NIH had intentionally, systematically, and mischievously used the eponym Creutzfeldt-Jakob disease, rather than Jakob-Creutzfeldt disease, because of the correspondence with Gibbs' own initials, to imply "Clarence Joseph disease." The present study examines temporal trends in the use of "Creutzfeldt-Jakob" and "Jakob-Creutzfeldt" in scientific articles and monographs from 1946 to 2019 to assess whether there was a "Clarence J. Gibbs effect" that influenced the general use of a specific eponym by the scientific community. During Gibbs' period of publication on Creutzfeldt-Jakob disease (CJD), there was an abrupt, dramatic, and steady increase in use of the CJD eponym while use of the Jakob-Creutzfeldt disease (JCD) eponym remained at a low level. In the period after Gibbs ceased to publish, there was a corresponding marked fall-off in use of the CJD eponym. Surviving collaborators thought Gibbs may have been joking, but in 1991 Gibbs had admitted what Prusiner reported. Regardless of motive, Gibbs strongly influenced the preferred eponym for this human prion disease by (1) publishing a seminal and highly referenced initial article in a high-profile journal; (2) sustained output of further important studies published in high-quality journals over more than 30 years; (3) professional affiliation with an esteemed national laboratory where he worked with a large number of high-profile colleagues; and (4) extensive collaborations with a large number of colleagues, who published multiple further articles using the eponym Gibbs preferred.

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The Last Shot

Gutmann, Ludwig MD

doi : 10.1212/WNL.0000000000011923

Volume 97(4), 27 July 2021, p 188-190

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Neuro-Behcet Disease Manifesting as Multiple Cerebral Aneurysms

Guimaraes, Filipa MD; Duque, Cristina MD

doi : 10.1212/WNL.0000000000012184

Volume 97(4), 27 July 2021, p 191-192

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Resident & Fellow Rounds

Strowd, Roy E. III MD, MEd, MS, FAAN; Editor; Aamodt, Whitley MD, MPH; Deputy Editor

doi : 10.1212/WNL.0000000000012332

Volume 97(4), 27 July 2021, p 193-195

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Pearls & Oy-sters: Idiopathic Normal Pressure Hydrocephalus With Synucleinopathy: Diagnosis and Treatment

Han, Sang-Won MD; Park, Young Ho MD, PhD; Ryoo, Nayoung MD; Kim, Kitae MD; Pyun, Jung-Min MD; Kim, SangYun MD, PhD

doi : 10.1212/WNL.0000000000012099

Volume 97(4), 27 July 2021, p 196-199

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Clinical Reasoning: A 68-Year-Old Man With Proximal Weakness and Seizures

Chen, Tychicus MD, FRCPC

doi : 10.1212/WNL.0000000000012137

Volume 97(4), 27 July 2021, p e423-e428

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Teaching Video NeuroImage: Clenched Fists as an Unusual Presentation of Focal Neuromyotonia

Dhanapalaratnam, Roshan MMed, MBBS; Schwartz, Raymond PhD, MBBS; Huynh, William PhD, MBBS, BSc

doi : 10.1212/WNL.0000000000012055

Volume 97(4), 27 July 2021, p e429-e430

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Teaching Video NeuroImage: ECG-Gated 4-D CT Angiography Can Detect Aortic Plaque Mobility in Cryptogenic Stroke

Saito, Mikito MD; Kawano, Hiroyuki MD, PhD; Koyanagi, Masamichi MSc; Gomyo, Miho MD, PhD; Yokoyama, Kenichi MD, PhD; Hirano, Teruyuki MD, PhD

doi : 10.1212/WNL.0000000000012054

Volume 97(4), 27 July 2021, p e431-e432

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Myasthenia Gravis: Which Type of Surgery Is Best?

Karceski, Steven MD

doi : 10.1212/WNL.0000000000012339

Volume 97(4), 27 July 2021, p e433-e435

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Editors' Note: Leadership, Recognition Awards, and Publication by Men and Women in the American Academy of Neurology

Lewis, Ariane MD; Galetta, Steven MD

doi : 10.1212/WNL.0000000000012337

Volume 97(4), 27 July 2021, p 200

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Reader Response: Leadership, Recognition Awards, and Publication by Men and Women in the American Academy of Neurology

Langer-Gould, Annette

doi : 10.1212/WNL.0000000000012333

Volume 97(4), 27 July 2021, p 200-201

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Author Response: Leadership, Recognition Awards, and Publication by Men and Women in the American Academy of Neurology

Keran, Chris; Gross, Robert A.; Yuan, Yan; Miyasaki, Janis M.

doi : 10.1212/WNL.0000000000012334

Volume 97(4), 27 July 2021, p 201

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Reader Response: Leadership, Recognition Awards, and Publication by Men and Women in the American Academy of Neurology

Silver, Julie K.; Bank, Anna M.; Poorman, Julie A.; Goldstein, Richard

doi : 10.1212/WNL.0000000000012335

Volume 97(4), 27 July 2021, p 201-202

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Author Response: Leadership, Recognition Awards, and Publication by Men and Women in the American Academy of Neurology

Gross, Robert A.; Yuan, Yan; Keran, Chris; Miyasaki, Janis M.

doi : 10.1212/WNL.0000000000012336

Volume 97(4), 27 July 2021, p 203

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Long-term Clinical Outcomes of Hematopoietic Stem Cell Transplantation in Multiple Sclerosis

doi : 10.1212/WNL.0000000000011825

Volume 97(4), 27 July 2021, p 203

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Hospital Admission and Readmission Among Homeless Patients With Neurologic Disease

doi : 10.1212/WNL.0000000000009565

Volume 97(4), 27 July 2021, p 203

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