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Spotlight on the November 15 Issue.
Merino, Jose G. MD, MPhil; Editor-in-Chief, Neurology(R)
doi : 10.1212/WNL.0000000000201360
Volume 99(20) pgs. 871-921,e2212-e2335 November 15, 2022
Inclusion Body Myositis: Boundaries That May Define Transition to Treatment Refractoriness.
Greenberg, Steven A. MD
Incomplete Enrollment in Clinical Trials: What Can We Learn?.
Hartman, Adam L. MD
Can CSF Biomarkers Decode Contributions of Combined A[beta] Plaque, Tau Tangle, and [alpha]-Synuclein Pathology?.
Knopman, David S. MD
Morphologic and Molecular Patterns of Polymyositis With Mitochondrial Pathology and Inclusion Body Myositis.
Kleefeld, Felix MD; Uruha, Akinori MD; Schanzer, Anne MD; Nishimura, Anna MD; Roos, Andreas PhD; Schneider, Udo MD; Goebel, Hans H. MD; Schuelke, Markus MD; Hahn, Katrin MD; Preusse, Corinna PhD; Stenzel, Werner MD
doi : 10.1212/WNL.0000000000201103
To characterize morphological and molecular underpinnings of polymyositis with mitochondrial pathology (PM-Mito) in comparison with sporadic inclusion body myositis (IBM) and to define common and distinct pathophysiologic features with a focus on interferon (IFN)-associated inflammation and T-cell response. Methods: In this cross-sectional study, skeletal muscle biopsy samples and clinical and laboratory data from patients with PM-Mito and IBM were analyzed at Charite university hospital in Berlin, Germany.
Neuromuscular Features in XL-MTM Carriers: A Cross-sectional Study in an Unselected Cohort.
Franken, Danielle K. BSc *; Bouman, Karlijn MD *; Reumers, Stacha F. I. MSc; Braun, Frederik MD, PhD; Spillane, Jennifer MD, PhD; Pennings, Maartje BSc; Houwen, Saskia L.S. MD; Erasmus, Corrie E. MD, PhD; Schara-Schmidt, Ulrike MD, PhD; Kamsteeg, Erik-Jan MSc; Jungbluth, Heinz MD, PhD; Voermans, Nicol C. MD, PhD
doi : 10.1212/WNL.0000000000201084
X-linked myotubular myopathy (XL-MTM) is an early-onset congenital myopathy characterized by mild to severe muscle weakness in male individuals. The objective was to characterize the clinical spectrum of neuromuscular features in X-linked myotubular myopathy (XL-MTM) carriers.
Prevalence and Risk Factor Profiles for Chronic Axonal Polyneuropathy in the General Population.
Taams, Noor E. MD; Drenthen, Judith MD; Hanewinckel, Rens MD, PhD; Ikram, M. Arfan MD, PhD; van Doorn, Pieter A. MD, PhD
doi : 10.1212/WNL.0000000000201168
Chronic axonal polyneuropathy is a common disease with increasing prevalence with age. It majorly affects quality of life and leads to difficulties with various activities. Persons with polyneuropathy often not seek medical care and thus the societal burden of disease is likely underreported. Given the aging populations, contemporary data on the prevalence and risk factor profiles of polyneuropathy in the general population are required.
Efficacy of Prednisolone for Bell Palsy in Children: A Randomized, Double-Blind, Placebo-Controlled, Multicenter Trial.
Babl, Franz E. MD; Herd, David MBChB; Borland, Meredith L. MBBS; Kochar, Amit MD; Lawton, Ben MBChB; Hort, Jason MBBS; West, Adam MHSM; George, Shane MBBS; Zhang, Michael PhD; Velusamy, Karthik MBBS; Sullivan, Frank PhD; Oakley, Ed MBBS; Davidson, Andrew MD; Hopper, Sandy M. MBBS; Cheek, John A. MBBS; Berkowitz, Robert G. MD; Hearps, Stephen MBiostat; Wilson, Catherine L. MPH; Williams, Amanda BNurs; Elborough, Hannah BSc (Hons); Legge, Donna BPharm; Mackay, Mark T. PhD; Lee, Katherine J. PhD; Dalziel, Stuart R. PhD; on behalf of Paediatric Research in Emergency Departments International Collaborative (PREDICT)
doi : 10.1212/WNL.0000000000201164
Corticosteroids are used to treat the early stages of idiopathic facial paralysis (Bell palsy) in children, but their effectiveness is uncertain. We set out to determine whether prednisolone improves the proportion of children with Bell palsy with complete recovery at 1 month.
Evaluation of Compensation Strategies for Gait Impairment in Patients With Parkinson Disease.
Tosserams, Anouk MD; Keijsers, Noel PhD; Kapelle, Willanka MD; Kessels, Roy P.C. PhD; Weerdesteyn, Vivian PhD; Bloem, Bastiaan R. MD, PhD; Nonnekes, Jorik
doi : 10.1212/WNL.0000000000201159
Compensation strategies are essential in Parkinson disease (PD) gait rehabilitation. However, besides external cueing, these strategies have rarely been investigated systematically. We aimed to perform the following: (1) establish the patients' perspective on the efficacy and usability of 5 different compensation strategies; (2) quantify the efficacy of these strategies on spatiotemporal gait parameters; and (3) explore associations between the effects of specific strategies and patient characteristics.
Association of AK4 Protein From Stem Cell-Derived Neurons With Cognitive Reserve: An Autopsy Study.
Yu, Lei PhD; Hsieh, Yi-Chen PhD; Pearse, Richard V. PhD; Wang, Yanling PhD; Petyuk, Vladislav A. PhD; Schneider, Julie A. MD; Buchman, Aron S. MD; Seyfried, Nicholas T. PhD; De Jager, Philip L. PhD; Young-Pearse, Tracy L. PhD; Bennett, David A. MD
doi : 10.1212/WNL.0000000000201120
Identifying protein targets that provide cognitive reserve is a strategy to prevent and treat Alzheimer disease and Alzheimer disease related dementias (AD/ADRD). Previous studies using bulk human brain tissue reported 12 proteins associated with cognitive reserve. This study examined whether the same proteins from induced neurons (iNs) are associated with cognitive reserve of their human donors.
Longitudinal Changes in Cognitive Functioning and Brain Structure in Professional Boxers and Mixed Martial Artists After They Stop Fighting.
Zhuang, Xiaowei MS; Bennett, Lauren PhD; Nandy, Rajesh PhD; Cordes, Dietmar PhD; Bernick, Charles MD *; Ritter, Aaron MD *
doi : 10.1212/WNL.0000000000201158
This study compares longitudinal changes in cognitive functioning and brain structures in male fighters who transitioned to an inactive fighting status without any further exposure to repetitive head impacts (RHIs) and fighters remaining active with continual exposure to RHIs.
Combined Effects of Synaptic and Axonal Integrity on Longitudinal Gray Matter Atrophy in Cognitively Unimpaired Adults.
Saloner, Rowan PhD; Fonseca, Corrina BS; Paolillo, Emily W. PhD; Asken, Breton M. PhD, ATC; Djukic, Nina A. BS; Lee, Shannon BS; Nilsson, Johanna MSc; Brinkmalm, Ann PhD; Blennow, Kaj MD, PhD; Zetterberg, Henrik MD, PhD; Kramer, Joel H. PsyD; Casaletto, Kaitlin B. PhD
doi : 10.1212/WNL.0000000000201165
Synaptic dysfunction and degeneration is a predominant feature of brain aging, and synaptic preservation buffers against Alzheimer disease (AD) protein-related brain atrophy. We tested whether CSF synaptic protein concentrations similarly moderate the effects of axonal injury, indexed by CSF neurofilament light [NfL]), on brain atrophy in clinically normal adults.
Association of Midlife Inflammatory Markers With Cognitive Performance at 10-Year Follow-up.
Kipinoinen, Teemu MD *; Toppala, Sini MD *; Rinne, Juha O. MD, PhD; Viitanen, Matti H. MD, PhD; Jula, Antti M. MD, PhD; Ekblad, Laura L. MD, PhD
doi : 10.1212/WNL.0000000000201116
Chronic low-grade inflammation, commonly associated with cardiovascular diseases and risk factors, has been associated inconclusively with cognitive decline and dementia. The aim of our study was to evaluate whether low-grade inflammation, measured in midlife, is associated with a decline in cognitive performance after a 10-year follow-up. We hypothesized that low-grade inflammation, estimated by interleukin-6 (IL-6), tumor necrosis factor alpha (TNF-[alpha]), and high-sensitivity CRP (hs-CRP), is a predictor of cognitive decline in the general population.
CSF Biomarkers of Alzheimer Disease in Patients With Concomitant [alpha]-Synuclein Pathology.
Cousins, Katheryn Alexandra Quilico PhD; Arezoumandan, Sanaz MD; Shellikeri, Sanjana PhD; Ohm, Daniel PhD; Shaw, Leslie M. PhD; Grossman, Murray MD, EdD; Wolk, David MD; McMillan, Corey T. PhD; Chen-Plotkin, Alice MD; Lee, Edward MD, PhD; Trojanowski, John Q. PhD; Zetterberg, Henrik MD, PhD; Blennow, Kaj MD, PhD; Irwin, David John MD
doi : 10.1212/WNL.0000000000201202
CSF biomarkers [beta]-amyloid 1-42 (A[beta]42), phosphorylated tau 181 (p-tau181), total tau (t-tau), and neurogranin (Ng) can diagnose Alzheimer disease (AD) in life. However, it is unknown whether CSF concentrations, and thus their accuracies, are affected by concomitant pathologies common in AD, such as [alpha]-synuclein ([alpha]Syn). Our primary goal was to test whether biomarkers in patients with AD are altered by concomitant [alpha]Syn.
Effect of Cholinesterase Inhibitors on Mortality in Patients With Dementia: A Systematic Review of Randomized and Nonrandomized Trials.
Truong, Celine MD; Recto, Caryn MD; Lafont, Charlotte MD; Canoui-Poitrine, Florence MD, PhD; Belmin, Joel Belmin MD, PhD; Lafuente-Lafuente, Carmelo MD, PhD
doi : 10.1212/WNL.0000000000201161
Cholinesterase inhibitors (ChEIs) have cardiovascular effects in addition to their neurologic activity and might alter mortality. We wanted to know whether treatment with ChEIs modifies mortality in patients with dementia. Methods: We searched PubMed, Embase, Cochrane CENTRAL, ClinicalTrials.gov, and ICRTP, from their inception to November 2021, and screened bibliographies of reviews, guidelines, and included studies.
Delirium in Adults With COVID-19-Related Acute Respiratory Distress Syndrome: Comparison With Other Etiologies.
Bernard-Valnet, Raphael MD, PhD; Favre, Eva RN, MScN; Bernini, Adriano PhD; Oddo, Mauro MD; Chiche, Jean-Daniel MD, PhD; Du Pasquier, Renaud A. MD; Rossetti, Andrea O. MD, FAES; on behalf of the CORO-NEURO-ICU Study Group
doi : 10.1212/WNL.0000000000201162
Neurologic complications have been associated with COVID-19, including delirium. Such complications have been reported to be frequent among intensive care unit (ICU)-admitted patients. We hypothesized that the rate of neurologic complications would be higher in COVID-19 associated acute respiratory distress syndrome (ARDS) than those who develop ARDS from a different cause.
What Is the Role of Dorsal Horn Astrocytes in Chronic Pain and Itch?.
Benarroch, Eduardo MD
Extracranial Etiology of Acute Onset Ataxia and Weakness: Small but Deadly.
Nordli, Douglas MD; Martz, Blake DO; Abram, Harry MD
MRI Features of Anti-Ma1/Ma2 Paraneoplastic Neurologic Syndrome.
Kunchok, Amy C. MBBS, MMed, FRACP; Ontaneda, Daniel MD, PhD; Lee, Jonathan MD; Rae-Grant, Alexander MD; Foldvary-Schaefer, Nancy DO; Cohen, Jeffrey A. MD; Jones, Stephen E. MD, PhD
Opinion & Special Article: Glioma Classification: How to Interpret Molecular Markers in a Diffuse Glioma Pathology Report.
van der Meulen, Matthijs MD, PhD; Ramos, Ronald C. MD; Mason, Warren P. MD; Von Deimling, Andreas MD, PhD; Maas, Sybren L.N. MD, PhD
doi : 10.1212/WNL.0000000000201262
AB Diffuse infiltrating gliomas are the most common malignant brain tumors in adults. The 2021 World Health Organization classification for CNS tumors (CNS5 WHO) has significantly altered the rules for classification and grading of diffuse gliomas. Clinicians, including neurology residents and neurologists, will have to consider the changes that include the introduction of new tumor types, allotting established tumor types to other groups and substituting previously essential morphological features for additional molecular markers. For example, in the current classification, glioblastoma is defined as isocitrate dehydrogenase (IDH)-wildtype, grade 4.
Pearls & Oy-sters: Managing Cholesterol in a Patient With Statin Intolerance Due to Anti-HMG-CoA Reductase-Associated Myopathy.
Villa, Eduardo Bachelor of Medicine; Naves, Rodrigo PhD; Bezares, Katiuska MD; Cobena, Karen MD; Villarroel, Ana Claudia MD; Guevara, Carlos PhD
doi : 10.1212/WNL.0000000000201315
AB Statins are the first line of treatment for hypercholesterolemia and of prevention of atherosclerotic cardiovascular disease (ASCVD). It is estimated that 1 in 4 Americans over the age of 40 years use statins. In rare cases, patients may develop an autoimmune myopathy associated with antibodies against 3-hydroxy-3-methylglutaryl coenzyme A reductase (HMGCR). Anti-HMGCR-associated myopathy requires immediate discontinuation of statins plus initiation of immunosuppressive therapy.
Teaching NeuroImage: Optic Pathway Involvement in Maple Syrup Urine Disease.
Narra, Rama Krishna MD, DNB, DM; Pamidi, Rajendra Kumar MD, DNB; Mandapalli, Adinarayana Rao MD
Teaching Video NeuroImage: Scurvy Presenting as Proximal Myopathy in a Young Boy.
Garg, Meenal MD
Editors' Note: Impact of C-Reactive Protein on Cognition and Alzheimer Disease Biomarkers in Homozygous APOE [Latin Small Letter Open E]4 Carriers.
Ganesh, Aravind MD, DPhil, FRCPC; Galetta, Steven MD
Reader Response: Impact of C-Reactive Protein on Cognition and Alzheimer Disease Biomarkers in Homozygous APOE [Latin Small Letter Open E]4 Carriers.
Royall, Donald R.
Author Response: Impact of C-Reactive Protein on Cognition and Alzheimer Disease Biomarkers in Homozygous APOE [Latin Small Letter Open E]4 Carriers.
Qiu, Wei Qiao; Tao, Qiushan; Akhter-Khan, Samia C.
Editors' Note: Relationship Between Headache Characteristics and a Remote History of TBI in Veterans: A 10-Year Retrospective Chart Review.
Ganesh, Aravind MD, DPhil, FRCPC; Galetta, Steven MD
Reader Response: Relationship Between Headache Characteristics and a Remote History of TBI in Veterans: A 10-Year Retrospective Chart Review.
Gupta, Vinod K.
Author Response: Reationship Between Headache Characteristics and a Remote History of TBI in Veterans: A 10-Year Retrospective Chart Review.
Androulakis, X. Michelle; Moore, R. Davis
