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Spotlight on the November 22 Issue.
Merino, Jose G. MD, MPhil; Editor-in-Chief, Neurology(R)
doi : 10.1212/WNL.0000000000201392
Volume 99(21) pgs. 923-969,e2336-e2427 November 22, 2022
Neurologists Have a Plan for Lifelong Brain Health.
Avitzur, Orly MD, MBA; Rost, Natalia S. MD, MPH; Evans, David A. MBA
Diabetes and Dementia: Spotlight on Multidomain Lifestyle Strategies.
Hooshmand, Babak MD, PhD, MPH; Solomon, Alina MD, PhD
Can We Generalize Key Principles in the Care of Rare Diseases?: The Case for Adrenoleukodystrophy.
Carlson, Martha D. MD, PhD; Kang, Peter B. MD
Association of Combined Healthy Lifestyle Factors With Incident Dementia in Patients With Type 2 Diabetes.
Wang, Bin MD, PhD *; Wang, Ningjian MD, PhD *; Sun, Ying MS; Tan, Xiao PhD; Zhang, Jihui MD, PhD; Lu, Yingli MD, PhD
doi : 10.1212/WNL.0000000000201231
Type 2 diabetes and lifestyle factors have been associated with dementia risk, but the effect of a healthy lifestyle on diabetes-related dementia remains largely unknown. We aimed to investigate whether the increased risk of dementia among individuals with diabetes can be offset by a broad combination of healthy lifestyle factors. Methods: This prospective study used data from the UK Biobank cohort.
Epilepsy, Vascular Risk Factors, and Cognitive Decline in Older Adults: The Cardiovascular Health Study.
Choi, Hyunmi MD, MS; Elkind, Mitchell S.V. MD; Longstreth, W.T. Jr MD; Boehme, Amelia K. PhD; Hafen, Rebekah BS; Hoyt, Emma J. MPH; Thacker, Evan L. PhD
doi : 10.1212/WNL.0000000000201187
Recent studies have shown that global cognitive ability tends to decline faster over time in older adults (>=65 years) with epilepsy compared with older adults without epilepsy. Scarce data exist about the role of vascular risk factors (VRFs) on cognitive course in epilepsy. We assessed whether the associations of individual VRFs with cognitive trajectory differed depending on the presence of prevalent epilepsy.
Epilepsy Risk Following Bariatric Surgery for Weight Loss.
Antaya, Tresah C. MPH; Qureshi, Amna MPH; Richard, Lucie MA; Shariff, Salimah Z. PhD; Elnahas, Ahmad MD, MSc; Burneo, Jorge G. MD, MSPH
doi : 10.1212/WNL.0000000000201100
A previous study reported finding that epilepsy risk is elevated after bariatric surgery for weight loss; however, this association has not been adequately explored. Our objectives were to (1) estimate the risk of epilepsy after bariatric surgery for weight loss relative to a nonsurgical cohort of patients with an obesity diagnosis and (2) identify epilepsy risk factors among bariatric surgery recipients.
Predictors of Recurrent Venous Thrombosis After Cerebral Venous Thrombosis: Analysis of the ACTION-CVT Study.
Shu, Liqi MD; Bakradze, Ekaterina MD; Omran, Setareh Salehi MD; Giles, James MD; Amar, Jordan MD; Henninger, Nils MD; Elnazeir, Marwa MD; Liberman, Ava MD; Moncrieffe, Khadean BS; Rotblat, Jenny MD; Sharma, Richa MD, MPH; Cheng, Yee MD; Zubair, Adeel S. MD; Simpkins, Alexis MD; Li, Grace MD; Kung, Justin; Perez, Dezaray MD; Heldner, Mirjam R. MD, MSc; Scutelnic, Adrian MD; von Martial, Rascha MD; Siepen, Bernhard MD; Rothstein, Aaron MD; Khazaal, Ossama MD; Do, David MD; Al Kasab, Sami MD; Rahman, Line Abdul MD; Mistry, Eva A. MD; Kerrigan, Deborah MD; Lafever, Hayden; Nguyen, Thanh N. MD; Klein, Piers; Aparicio, Hugo J. MD, MPH; Frontera, Jennifer A. MD; Kuohn, Lindsey BS; Agarwal, Shashank MD; Stretz, Christoph MD; Kala, Narendra MD; ElJamal, Sleiman MD; Chang, Allison; Cutting, Shawna MD; Indraswari, Fransisca MD; de Havenon, Adam MD; Muddasani, Varsha MD; Wu, Teddy MD; Wilson, Duncan PhD; Nouh, Amre MD; Asad, Daniyal MD; Qureshi, Abid MD; Moore, Justin MD; Khatri, Pooja MD; Aziz, Yasmin MD; Casteigne, Bryce MD; Khan, Muhib MD; Cheng, Yao MD; Grory, Brian Mac MD; Weiss, Martin MD; Ryan, Dylan MD; Vedovati, Maria Cristina MD; Paciaroni, Maurizio MD; Siegler, James MD; Kamen, Scott MD; Yu, Siyuan MD; Guerrero, Christopher Leon MD; Atallah, Eugenie MD; De Marchis, Gian Marco MD; Brehm, Alex MD; Dittrich, Tolga MD; Psychogios, Marios MD; Alvarado-Dyer, Ronald MD; Kass-Hout, Tareq MD; Prabhakaran, Shyam MD; Honda, Tristan MD; Liebeskind, David MD; Furie, Karen MD; Yaghi, Shadi MD
doi : 10.1212/WNL.0000000000201122
Cerebral venous thrombosis (CVT) is a rare cause of stroke carrying a nearly 4% risk of recurrence after 1 year. There are limited data on predictors of recurrent venous thrombosis in patients with CVT. In this study, we aim to identify those predictors.
Stroke Symptoms as a Surrogate in Stroke Primary Prevention Trials: The CREST Experience.
Meschia, James F. MD; Brott, Thomas G. MD; Voeks, Jenifer PhD; Howard, Virginia J. PhD; Howard, George DrPH
doi : 10.1212/WNL.0000000000201188
The use of surrogate end points can decrease sample size while maintaining statistical power. This report considers incident stroke symptoms as a surrogate end point in a post hoc analysis of asymptomatic patients from the multicenter, randomized Carotid Revascularization Endarterectomy vs Stenting Trial (CREST).
Association of Contrast Enhancement After Reperfusion With Outcomes According to Blood Pressure Lowering in Patients With Acute Ischemic Stroke.
Maier, Benjamin MD, MSc *; Brauner, Ran MD *; Escalard, Simon MD; Gory, Benjamin MD, PhD; Lapergue, Bertrand MD, PhD; Sibon, Igor MD, PhD; Richard, Sebastien MD, PhD; Labreuche, Julien MSc; Kyheng, Maeva PhD; Desilles, Jean-Philippe MD, PhD; Blanc, Raphael MD, MSc; Piotin, Michel MD, PhD; Halimi, Jean-Michel MD, PhD; Mazighi, Mikael MD, PhD; on behalf of the BP-TARGET Trial Investigators
doi : 10.1212/WNL.0000000000201173
Observational studies described associations between higher systolic blood pressure (SBP) values and intracranial hemorrhages (ICHs) and worse outcomes after successful reperfusion by endovascular therapy (EVT). However, the BP-TARGET trial [BP-Target in Acute Ischemic Stroke to Reduce Hemorrhage after EVT] found that an intensive SBP target did not reduce ICH rates after successful EVT.
Flexibility of Fast Brain Dynamics and Disease Severity in Amyotrophic Lateral Sclerosis.
Polverino, Arianna PhD *; Troisi Lopez, Emahnuel PhD *; Minino, Roberta PhD; Liparoti, Marianna PhD; Romano, Antonella PhD; Trojsi, Francesca MD, PhD; Lucidi, Fabio PhD; Gollo, Leonardo PhD; Jirsa, Viktor PhD; Sorrentino, Giuseppe MD, PhD; Sorrentino, Pierpaolo PhD, MD
doi : 10.1212/WNL.0000000000201200
Amyotrophic lateral sclerosis (ALS) is a multisystem disorder, as supported by clinical, molecular, and neuroimaging evidence. As a consequence, predicting clinical features requires a description of large-scale neuronal dynamics.
Evaluating Genetic Modifiers of Duchenne Muscular Dystrophy Disease Progression Using Modeling and MRI.
Barnard, Alison M. DPT, PhD; Hammers, David W. PhD; Triplett, William T. BS; Kim, Sarah PhD; Forbes, Sean C. PhD; Willcocks, Rebecca J. PhD; Daniels, Michael J. ScD; Senesac, Claudia R. PT, PhD; Lott, Donovan J. PT, PhD; Arpan, Ishu PT, PhD; Rooney, William D. PhD; Wang, Richard T. PhD; Nelson, Stanley F. MD; Sweeney, H. Lee PhD; Vandenborne, Krista PT, PhD; Walter, Glenn A. PhD
doi : 10.1212/WNL.0000000000201163
Duchenne muscular dystrophy (DMD) is a progressive muscle degenerative disorder with a well-characterized disease phenotype but considerable interindividual heterogeneity that is not well understood.
Disease-Associated [alpha]-Synuclein Aggregates as Biomarkers of Parkinson Disease Clinical Stage.
Majbour, Nour PhD; Aasly, Jan MD; Abdi, Ilham MSc; Ghanem, Simona PhD; Erskine, Daniel PhD; van de Berg, Wilma PhD; El-Agnaf, Omar PhD
doi : 10.1212/WNL.0000000000201199
Robust biomarkers that can mirror Parkinson disease (PD) are of great significance. In this study, we present a novel approach to investigate disease-associated [alpha]-synuclein ([alpha]Syn) aggregates as biomarkers of PD clinical stage.
International Recommendations for the Diagnosis and Management of Patients With Adrenoleukodystrophy: A Consensus-Based Approach.
Engelen, Marc MD, PhD; van Ballegoij, Wouter J.C. MD, PhD; Mallack, Eric James MD; Van Haren, Keith P. MD; Kohler, Wolfgang MD; Salsano, Ettore MD; van Trotsenburg, A.S.P. MD, PhD; Mochel, Fanny MD, PhD; Sevin, Caroline MD, PhD; Regelmann, Molly O. MD; Tritos, Nicholas A. MD; Halper, Alyssa MD; Lachmann, Robin H. PhD; Davison, James PhD; Raymond, Gerald V. MD; Lund, Troy C. MD, PhD; Orchard, Paul J. MD; Kuehl, Joern-Sven MD; Lindemans, Caroline A. MD, PhD; Caruso, Paul MD; Turk, Bela Rui MD; Moser, Ann B. BA; Vaz, Frederic M. PhD; Ferdinandusse, Sacha PhD; Kemp, Stephan PhD; Fatemi, Ali MD; Eichler, Florian S. MD; Huffnagel, Irene C. MD, PhD
doi : 10.1212/WNL.0000000000201374
AB Pathogenic variants in the ABCD1 gene cause adrenoleukodystrophy (ALD), a progressive metabolic disorder characterized by 3 core clinical syndromes: a slowly progressive myeloneuropathy, a rapidly progressive inflammatory leukodystrophy (cerebral ALD), and primary adrenal insufficiency. These syndromes are not present in all individuals and are not related to genotype.
Bridging the Gap: Gene Therapy in a Patient With Spinal Muscular Atrophy Type 1.
Costamagna, Gianluca MD; Govoni, Alessandra MD; Wise, Adina MD; Corti, Stefania MD, PhD
doi : 10.1212/WNL.0000000000201294
AB Molecular therapies exploit the understanding of pathogenic mechanisms to reconstitute impaired gene function or manipulate flawed RNA expression. These therapies include (1) RNA interference by antisense oligonucleotides, (2) mRNA modification using small molecules, and (3) gene replacement therapy, the viral-mediated intracellular delivery of exogenous nucleic acids to reverse a genetic defect. Several molecular therapies are approved for treating spinal muscular atrophy (SMA), a recessive genetic disorder caused by survival motor neuron (SMN)1 gene alterations.
Pearls & Oy-sters: Deep Phenotyping of Abnormal Eye Movements Advances the Detection of Gerstmann-Straussler-Scheinker Syndrome.
Paul, Ashley M. MD; Mu, Weiyi ScM; Butala, Ankur MD *; Green, Kemar E. DO *
doi : 10.1212/WNL.0000000000201321
AB A 58-year-old previously healthy woman presents with 3 years of rapidly progressive ataxia, parkinsonism, dysautonomia, peripheral neuropathy, leg weakness, spasticity, hyperreflexia, and mild vertical-gaze palsy. She has a matrilineal family history of neurodegenerative diseases. She was initially postulated to have spinocerebellar ataxia or atypical parkinsonism with cerebellar features. However, on closer inspection, her abnormal extraocular eye movements suggested rare mimicking disorders such as prion disease as part of the differential diagnosis, requiring further evaluation.
Teaching NeuroImage: Absence of Wrinkles in Small Fiber Neuropathy.
Piedrafita Vico, Lucas Augusto MD; Reisin, Ricardo MD; Gonorazky, Sergio MD
Teaching NeuroImage: Occipital Cortical Enhancement During Encephalopathy-like Episode in Neuronal Intranuclear Inclusion Disease.
Wu, Jibao MD; Li, Qiuli MD; Yi, Jiping MD, PhD
Editors' Note: Oral and Topical Treatment of Painful Diabetic Polyneuropathy: Practice Guideline Update Summary: Report of the AAN Guideline Subcommittee.
Lewis, Ariane MD; Galetta, Steven MD, FAAN
Reader Response: Oral and Topical Treatment of Painful Diabetic Polyneuropathy: Practice Guideline Update Summary: Report of the AAN Guideline Subcommittee.
Argoff, Charles E.
Author Response: Oral and Topical Treatment of Painful Diabetic Polyneuropathy: Practice Guideline Update Summary: Report of the AAN Guideline Subcommittee.
Callaghan, Brian C.
Editors' Note: Poststroke Seizures and the Risk of Dementia Among Young Stroke Survivors.
Lewis, Ariane MD; Galetta, Steven MD, FAAN
Reader Response: Poststroke Seizures and the Risk of Dementia Among Young Stroke Survivors.
Feyissa, Anteneh M.; Freund, Brin E.
Author Response: Poststroke Seizures and the Risk of Dementia Among Young Stroke Survivors.
Lekoubou, Alain
