Neurology

Simpson-Yap, Steve PhD, MPH*; De Brouwer, Edward MSc*; Kalincik, Tomas MD, PhD, PGCertBiostat; Rijke, Nick MA; Hillert, Jan A. MD, PhD; Walton, Clare PhD; Edan, Gilles MD; Moreau, Yves PhD; Spelman, Tim PhD; Geys, Lotte PhD; Parciak, Tina MSc; Gautrais, Clement PhD; Lazovski, Nikola PDEng; Pirmani, Ashkan MSc; Ardeshirdavanai, Amin PhD; Forsberg, Lars MSc; Glaser, Anna PhD; McBurney, Robert PhD; Schmidt, Hollie MS; Bergmann, Arnfin B. MD, PhD; Braune, Stefan PhD; Stahmann, Alexander MSc; Middleton, Rodden MBA; Salter, Amber PhD; Fox, Robert J. MD; van der Walt, Anneke PhD; Butzkueven, Helmut PhD; Alroughani, Raed PhD; Ozakbas, Serkan PhD; Rojas, Juan I. MSc; van der Mei, Ingrid PhD; Nag, Nupur PhD; Ivanov, Rumen MSc; Sciascia do Olival, Guilherme PhD; Dias, Alice Estavo PhD; Magyari, Melinda PhD; Brum, Doralina PhD; Mendes, Maria Fernanda PhD; Alonso, Ricardo N. MD, MSc; Nicholas, Richard S. PhD; Bauer, Johana PhD; Chertcoff, Anibal Sebastian MD; Zabalza, Anna MD; Arrambide, Georgina MD, PhD; Fidao, Alexander MPH; Comi, Giancarlo MD+; Peeters, Liesbet PhD+

doi : 10.1212/WNL.0000000000012753

Volume 97(19), 9 November 2021, p e1870-e1885

People with multiple sclerosis (MS) are a vulnerable group for severe coronavirus disease 2019 (COVID-19), particularly those taking immunosuppressive disease-modifying therapies (DMTs). We examined the characteristics of COVID-19 severity in an international sample of people with MS.

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Mechanisms of Network Changes in Cognitive Impairment in Multiple Sclerosis

Jandric, Danka MSc*; Lipp, Ilona PhD*; Paling, David MRCP, PhD; Rog, David MD; Castellazzi, Gloria PhD; Haroon, Hamied PhD; Parkes, Laura PhD; Parker, Geoff J.M. PhD; Tomassini, Valentina MD, PhD+; Muhlert, Nils PhD+

doi : 10.1212/WNL.0000000000012834

Volume 97(19), 9 November 2021, p e1886-e1897

Cognitive impairment in multiple sclerosis (MS) is associated with functional connectivity abnormalities. While there have been calls to use functional connectivity measures as biomarkers, there remains to be a full understanding of why they are affected in MS. In this cross-sectional study, we tested the hypothesis that functional network regions may be susceptible to disease-related "wear and tear" and that this can be observable on co-occurring abnormalities on other magnetic resonance metrics. We tested whether functional connectivity abnormalities in cognitively impaired patients with MS co-occur with (1) overlapping, (2) local, or (3) distal changes in anatomic connectivity and cerebral blood flow abnormalities.

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Serum Neurofilament Light Association With Progression in Natalizumab-Treated Patients With Relapsing-Remitting Multiple Sclerosis

Bridel, Claire MD, PhD; Leurs, Cyra E. MD, PhD; van Lierop, Zoe Y.G.J. MD, PhD; van Kempen, Zoe L.E. MD, PhD; Dekker, Iris MD, PhD; Twaalfhoven, Harry A.M.; Moraal, Bastiaan MD, PhD; Barkhof, Frederik MD, PhD; Uitdehaag, Bernard M.J. MD, PhD; Killestein, Joep MD, PhD; Teunissen, Charlotte E. PhD

doi : 10.1212/WNL.0000000000012752

Volume 97(19), 9 November 2021, p e1898-e1905

To investigate the potential of serum neurofilament light (NfL) to reflect or predict progression mostly independent of acute inflammatory disease activity in patients with relapsing-remitting multiple sclerosis (RRMS) treated with natalizumab.

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Epidemiology, Clinical Features, and Outcome in a Cohort of Adolescents With Cerebral Venous Thrombosis

Devianne, Julia MD; Legris, Nicolas MD; Crassard, Isabelle MD; Bellesme, Celine MD; Bejot, Yannick MD; Guidoux, Celine MD; Pico, Fernando MD; Germanaud, David MD; Obadia, Michael MD; Rodriguez, Diana MD; Tuppin, Philippe MD; Kossorotoff, Manoelle MD; Denier, Christian MD, PhD

doi : 10.1212/WNL.0000000000012828

Volume 97(19), 9 November 2021, p e1920-e1932

We aimed to analyze the epidemiologic, clinical, and paraclinical features of adolescents with cerebral venous thrombosis (CVT) and its therapeutic management and outcome.

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Epidemiology of Treated Epilepsy in New Zealand Children: A Focus on Ethnicity

Ali, Shayma BSc; Stanley, James PhD; Davis, Suzanne MBChB, PhD; Keenan, Ngaire MBChB; Scheffer, Ingrid Eileen MBChB, PhD*; Sadleir, Lynette Grant MBChB, MD*

doi : 10.1212/WNL.0000000000012784

Volume 97(19), 9 November 2021, p e1933-e1941

To determine the period prevalence and incidence of treated epilepsy in a New Zealand pediatric cohort with a focus on ethnicity and socioeconomic status.

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Long-term Risks of Persistent Ventral Spinal CSF Leaks in SIH: Superficial Siderosis and Bibrachial Amyotrophy

Schievink, Wouter I. MD; Maya, Marcel MD; Moser, Franklin MD, MMM; Nuno, Miriam PhD

doi : 10.1212/WNL.0000000000012786

Volume 97(19), 9 November 2021, p e1964-e1970

Superficial siderosis, bibrachial amyotrophy, and spinal cord herniation are unusual but serious long-term sequelae of persistent spontaneous spinal CSF leaks in patients with spontaneous intracranial hypotension (SIH), particularly ventral spinal CSF leaks. However, the risk of developing such sequelae has not been established in this population. We undertook this study to determine the risk of these serious complications of persistent ventral spinal CSF leaks.

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Neurologic Diagnostics in 2035: The Neurology Future Forecasting Series

Ciccarelli, Olga PhD*; Pandolfo, Massimo MD*

doi : 10.1212/WNL.0000000000012833

Volume 97(19), 9 November 2021, p 902-907

Innovations and advances in technologies over the past few years have yielded faster and wider diagnostic applications to patients with neurologic diseases. This article focuses on the foreseeable developments of the diagnostic tools available to the neurologist in the next 15 years. Clinical judgment is and will remain the cornerstone of the diagnostic process, assisted by novel technologies, such as artificial intelligence and machine learning. Future neurologists must be educated to develop, cultivate, and rely on their clinical skills, while becoming familiar with novel, often complex, assistive technologies.

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Progressive Auditory Verbal Agnosia Secondary to Alzheimer Disease

Buciuc, Marina MD; Josephs, Keith Anthony MD; Jones, David T. MD; Whitwell, Jennifer L. PhD; Graff-Radford, Jonathan MD

doi : 10.1212/WNL.0000000000012783

Volume 97(19), 9 November 2021, p 908-909

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Clinical Reasoning: A Middle-aged Man With a History of Muscle Pain Presenting With Progressive Leukoencephalopathy and Subsequent Coma

Jakobsson, Anna Sofie MD; Strange, Ditte Gry MD; Moller, Kirsten MD, PhD; Kondziella, Daniel MD, PhD

doi : 10.1212/WNL.0000000000012486

Volume 97(19), 9 November 2021, p 910-915

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Opinion & Special Article: Preventive Neurology: An Emerging Field Toward Brain Health

Sabayan, Behnam MD, PhD; Isaacson, Richard MD; Rost, Natalia MD, MPH

doi : 10.1212/WNL.0000000000012554

Volume 97(19), 9 November 2021, p 916-919

With the rapid aging of populations, neurologic disorders have become among the leading causes of disability and mortality worldwide. Most neurologic conditions have a prolonged prodromal phase-even if they tend to manifest with an acute syndrome such as stroke-and can lead to a relentless, often deleterious course creating a major burden on patients, caregivers, and society. This unique nature of neurologic diseases signifies the strong need for equally effective primary and secondary prevention strategies and focus on brain health before brain diseases ensue. The field of preventive neurology applies both universal and selective primary prevention strategies to promote brain health both at the public and personal levels. The preventive neurology approach aims to identify and target high-risk individuals and protect them from reaching a critical point where overt clinical symptoms are present and disease progression is irreversible. Universal and selective prevention training, along with dovetailed clinical and public health research, are 3 essential pillars of preventive neurology. The burgeoning field of preventive neurology aims to assess neurologic care needs in a society, promote the participation of neurologists in restructuring of the health care policies to promote brain health, and identify medium- and high-risk individuals to prevent or delay future neurologic events.

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Teaching NeuroImage: Central Pontine Myelinolysis in Diabetic Ketoacidosis

Gonzalez Caldito, Natalia MD; Karim, Nurose MD; Gebreyohanns, Mehari MD

doi : 10.1212/WNL.0000000000012301

Volume 97(19), 9 November 2021, p e1971-e1972

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Teaching NeuroImage: Fluorosis: A Forgotten Cause of Compressive Myelopathy

Saluja, Alvee MBBS, MD, DM; Dhamija, Rajinder K. MBBS, MD, DNB, FRACP; Solanki, Ravi S. MBBS, MD

doi : 10.1212/WNL.0000000000012389

Volume 97(19), 9 November 2021, p e1973-e1974

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Editors' Note: Teaching NeuroImages: A Ruptured Lumbar Disc Mimicking Spinal Tumor

Siegler, James E. III MD; Galetta, Steven MD

doi : 10.1212/WNL.0000000000012835

Volume 97(19), 9 November 2021, p 920

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Reader Response: Teaching NeuroImages: A Ruptured Lumbar Disc Mimicking Spinal Tumor

Beucler, Nathan; Sellier, Aurore; Dagain, Arnaud

doi : 10.1212/WNL.0000000000012838

Volume 97(19), 9 November 2021, p 920-921

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Author Response: Teaching NeuroImages: A Ruptured Lumbar Disc Mimicking Spinal Tumor

Lee, Jung-Shun; Wong, Chia-En; Lee, Po-Hsuan; Huang, Chi-Chen; Chen, Hui-Wen; Tien, Chih-Hao; Huang, Chih-Yuan

doi : 10.1212/WNL.0000000000012837

Volume 97(19), 9 November 2021, p 921

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Editors' Note: Clinical Reasoning: A 70-Year-Old Man With Right Arm and Leg Shaking

Siegler, James E. III MD; Galetta, Steven MD

doi : 10.1212/WNL.0000000000012840

Volume 97(19), 9 November 2021, p 921-922

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Author Response: Clinical Reasoning: A 70-Year-Old Man With Right Arm and Leg Shaking

Frey, Jessica E.; Seachrist, Eric

doi : 10.1212/WNL.0000000000012842

Volume 97(19), 9 November 2021, p 923

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Comment: Introducing Neurologic Diagnostics in 2035

Jones, Lyell K. Jr MD; Gross, Robert A. MD, PhD

doi : 10.1212/WNL.0000000000012849

Volume 97(19), 9 November 2021, p 901

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Reader Response: Clinical Reasoning: A 70-Year-Old Man With Right Arm and Leg Shaking

Freund, Brin E.; Tatum, William O.

doi : 10.1212/WNL.0000000000012841

Volume 97(19), 9 November 2021, p 922-923

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Clinical Correlation of Multiple Sclerosis Immunopathologic Subtypes

Tobin, W. Oliver MB, BCh, BAO, PhD; Kalinowska-Lyszczarz, Alicja MD, PhD; Weigand, Stephen D. BS; Guo, Yong MD, PhD; Tosakulwong, Nirubol BS; Parisi, Joseph E. MD; Metz, Imke MD; Frischer, Josa M. MD, PhD; Lassmann, Hans MD; Bruck, Wolfgang MD; Linbo, Linda RN; Lucchinetti, Claudia F. MD

doi : 10.1212/WNL.0000000000012782

Volume 97(19), 9 November 2021, p e1906-e1913

The goal of this work was to compare clinical characteristics across immunopathologic subtypes of patients with multiple sclerosis.

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Prestroke Disability and Outcome After Thrombectomy for Emergent Anterior Circulation Large Vessel Occlusion Stroke

de Havenon, Adam MD; Castonguay, Alicia PhD; Nogueira, Raul MD; Nguyen, Thanh N. MD; English, Joey MD; Satti, Sudhakar Reddy MD; Veznedaroglu, Erol MD; Saver, Jeffrey L. MD, MBA; Mocco, J. MD; Khatri, Pooja MD; Mistry, Eva MBBS; Zaidat, Osama O. MD

doi : 10.1212/WNL.0000000000012827

Volume 97(19), 9 November 2021, p e1914-e1919

To determine the impact of endovascular therapy for large vessel occlusion stroke in patients with vs those without premorbid disability.

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Systematic Analysis of Brain MRI Findings in Adaptor Protein Complex 4-Associated Hereditary Spastic Paraplegia

Ebrahimi-Fakhari, Darius MD, PhD; Alecu, Julian E.; Ziegler, Marvin; Geisel, Gregory BS; Jordan, Catherine BA; D'Amore, Angelica PhD; Yeh, Rebecca C. BS; Akula, Shyam K. BA; Saffari, Afshin MD; Prabhu, Sanjay P. MD; Sahin, Mustafa MD, PhD; Yang, Edward MD, PhD; on behalf of the International AP-4-HSP Registry and Natural History Study; on behalf of the International AP-4-HSP Registry and Natural History Study; Bulk, Saskia MD, PhD; Depierreux, Fr[spacing acute]ed[spacing acute]erique MD; Habibzadeh, Parham MD; Iyer, Anand S. MD; Kaminska, Margaret MD; Kim, Soyoung MD; King, Staci D. MD; Mierzewska, Hanna MD, PhD; Roubertie, Agathe MD, PhD; Santorelli, Filippo M. MD, PhD; Shoukier, Moneef MD; Szczaluba, Krzysztof MD; Uysal, Sanem P. MD; van Ravenswaaij-Arts, Conny M.A. MD, PhD

doi : 10.1212/WNL.0000000000012836

Volume 97(19), 9 November 2021, p e1942-e1954

AP-4-associated hereditary spastic paraplegia (AP-4-HSP: SPG47, SPG50, SPG51, SPG52) is an emerging cause of childhood-onset hereditary spastic paraplegia and mimic of cerebral palsy. This study aims to define the spectrum of brain MRI findings in AP-4-HSP and to investigate radioclinical correlations.

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Life Course of Physical Activity and Risk and Prognosis of Amyotrophic Lateral Sclerosis in a German ALS Registry

Rosenbohm, Angela MD; Peter, Raphael PhD; Dorst, Johannes MD; Kassubek, Jan MD; Rothenbacher, Dietrich MD; Nagel, Gabriele MD*; Ludolph, Albert C. MD*; on behalf of the ALS Registry Swabia Study Group; on behalf of the ALS Registry Swabia Study Group; Andres, Frank MD; Arnold, Guy MD; Baier, Hartmut MD; Baezner, Hansjoerg MD; Beattie, James MD; Behne, Friedrich MD; Bengel, Dietmar MD; Boertlein, Axel MD; Vera, Bracknies MD; Alber, Burkhard MD; Buttmann, Mathias MD; Dempewolf, Silke MD; Dettmers, Christian MD; Freund, Wolfgang MD; Gasser, Thomas MD; Gold, Hans-Juergen MD; Hamann, Gerhard MD; Hecht, Martin MD; Heimbach, Bernhard MD; Herting, Birgit MD; Huber, Roman MD; Huelser, Paul-Juergen MD; HuberHartmann, Karlheinz MD; Juttler, Eric MD; Kaspar, Attila MD; Kern, Rolf MD; Kimmig, Hubert MD; Christof, Klotzsch MD; Chatzikonstantinou, Anastasios MD; Klopstock, Thomas MD; Kohler, Andreas MD; Lichy, Christoph MD; Lindner, Alfred MD; Lingor, Paul MD; Lule, Dorothee PhD; Metrikat, Jens MD; Meudt, Oliver MD; Meyer, Andreas MD; Naegele, Andrea MD; Naumann, Markus MD; Neher, Klaus-Dieter MD; Neuhaus, Oliver MD; Neusch, Clemens MD; Niehaus, Ludwig MD; Raape, Jan MD; Ratzka, Peter MD; Reinhard, Matthias MD; Rothmeier, Johann MD; Sabolek, Michael MD; Schabet, Martin; SchaeffVogelsang, MDMario MD; Schell, Caroline MD; Schell, Caroline MD; Schweigert, Barbara MD; Sommer, Norbert MD; Stroick, Mark MD; Synofzik, Mathis MD, ThomasTrottenberg, MD; Tumani, Hayrettin MD; Volkmann, Jens MD; Weiler, Markus MD; Wick, Wolfgang MD; Opherk, Christian MD; Weiller, Cornelius MD; Zeller, Daniel MD; Baumgartner, Jessica MD; Born, Christoph MD; Burgy, Martin MD; Connemann, Bernhard MD; Etzersdorfer, Elmar MD; Friederich, Hubertus MD; Gahr, Maximilian MD; Gogolkiewicz, Alex MD; Greber, Ralf MD; Gebhardt, Jochen MD; Grunze, Heinz MD; Henkel, Karsten MD; Hewer, Walter MD; Raether, Andreas MD; Joos, Andreas MD; Kohler, Matthias MD; Kozian, Ralf MD; Laske, Christoph MD; Michaelides, Alexandros MD; Munk, Matthias MD; Niestroj, Andreas MD; Schmauss, Max MD; Schmauss, Max MD; SchoenebergerStroick, Katrin MD; Schoerner, Kai-Uwe MD; Spannhorst, Stefan MD; Steber, Raimund MD; Thomas, Christine MD; Vasic, Nenad MD

doi : 10.1212/WNL.0000000000012829

Volume 97(19), 9 November 2021, p e1955-e1963

Whether physical activity (PA) is a risk factor for amyotrophic lateral sclerosis (ALS) is controversial because data on lifelong PA are rare. The main objective of this study is to provide insight into PA as a potential risk factor for ALS, reporting data on cumulative PA, leisure-time PA, and occupational PA. This study also aims to gather evidence on the role of PA as a prognostic factor in disease course.

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