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Spotlight on the August 3 Issue
Merino, Jose G. MD, MPhil; Editor-in-Chief, Neurology(R)
doi : 10.1212/WNL.0000000000012361
Volume 97(5), 3 August 2021, p 205-206
Silent Myocardial Infarction and Stroke Risk: We Should Be Listening
Chaturvedi, Seemant MD; Chimowitz, Marc MBChB
doi : 10.1212/WNL.0000000000012248
Volume 97(5), 3 August 2021, p 207-208
Silent Myocardial Infarction and Subsequent Ischemic Stroke in the Cardiovascular Health Study
Merkler, Alexander E. MD, MS; Bartz, Traci M. MS; Kamel, Hooman MD; Soliman, Elsayed Z. MD, MSc, MS; Howard, Virginia PhD; Psaty, Bruce M. MD, PhD; Okin, Peter M. MD; Safford, Monika M. MD; Elkind, Mitchell S.V. MD, MS; Longstreth, W.T. Jr. MD, MPH
doi : 10.1212/WNL.0000000000012249
Volume 97(5), 3 August 2021, p e436-e443
To test the hypothesis that silent myocardial infarction (MI) is a risk factor for ischemic stroke, we evaluated the association between silent MI and subsequent ischemic stroke in the Cardiovascular Health Study.
Impact of Repeated Clot Retrieval Attempts on Infarct Growth and Outcome After Ischemic Stroke
Ben Hassen, Wagih MD*; Touloupas, Caroline MD*; Benzakoun, Joseph MD; Boulouis, Gregoire MD; Bretzner, Martin MD; Bricout, Nicolas MD; Legrand, Laurence MD; Rodriguez, Christine MD; Le Berre, Alice MD; Seners, Pierre MD, PhD; Turc, Guillaume MD, PhD; Cordonnier, Charlotte MD, PhD; Oppenheim, Catherine MD, PhD; Henon, Hilde MD, PhD+; Naggara, Olivier MD, PhD+
doi : 10.1212/WNL.0000000000012321
Volume 97(5), 3 August 2021, p e444-e453
To determine whether the association between increasing number of clot retrieval attempts (CRA) and unfavorable outcome is due to an increase in emboli to new territory (ENT) and greater infarct growth (IG) in successfully recanalized patients with acute ischemic stroke due to large vessel occlusion (AIS-LVO).
Enterovirus Meningitis in Adults: A Prospective Nationwide Population-Based Cohort Study
Bodilsen, Jacob MD, PhD; Mens, Helene MD, PhD; Midgley, Sofie MD; Brandt, Christian Thomas MD, DMSc; Petersen, Pelle Trier MD; Larsen, Lykke MD; Hansen, Birgitte Ronde MD, PhD; Luttichau, Hans Rudolf MD, DMSc; Helweg-Larsen, Jannik MD, DMSc; Wiese, Lothar MD, PhD; Ostergaard, Christian MD, DMSc; Storgaard, Merete MD; Nielsen, Henrik MD, PhD; and the Danish Study Group of Infections of the Brain (DASGIB)
doi : 10.1212/WNL.0000000000012294
Volume 97(5), 3 August 2021, p e454-e463
To test the hypothesis that enterovirus meningitis (EM) is a frequent and self-limiting condition, the epidemiology of EM in adults was examined.
Psychiatric Disorders in Children and Adolescents With Psychogenic Nonepileptic Seizures
Hansen, Anne Sofie MD; Rask, Charlotte Ulrikka MD, PhD; Christensen, Ann-Eva PhD; Rodrigo-Domingo, Maria PhD; Christensen, Jakob MD, PhD, DrMedSci; Nielsen, Rene Ernst MD, PhD
doi : 10.1212/WNL.0000000000012270
Volume 97(5), 3 August 2021, p e464-e475
Knowledge regarding psychiatric disorders in children and adolescents with psychogenic nonepileptic seizures (PNES) is limited. This study outlines the spectrum and risk of psychiatric disorders in childhood-onset PNES.
Measurement of Narcolepsy Symptoms in School-Aged Children and Adolescents: The Pediatric Narcolepsy Severity Scale
Barateau, Lucie MD, PhD; Lecendreux, Michel MD; Chenini, Sofiene MD; Rassu, Anna Laura MD; Lopez, Regis MD, PhD; Pesenti, Carole MSc; Jaussent, Isabelle PhD; Beziat, Severine MSc; Dauvilliers, Yves MD, PhD
doi : 10.1212/WNL.0000000000012272
Volume 97(5), 3 August 2021, p e476-e488
We validated the Narcolepsy Severity Scale (NSS) in adults with narcolepsy type 1 (NT1) to quantify the severity, frequency, and consequences of the 5 key narcolepsy symptoms over the last month, and we now developed the Pediatric NSS (NSS-P); thus, the aims of this study were to assess NSS-P psychometric properties, validity, and reliability, and to evaluate its responsiveness to treatment in a well-characterized sample of children and adolescents with NT1.
MicroRNAs as Biomarkers of Charcot-Marie-Tooth Disease Type 1A
Wang, Hongge MS; Davison, Matthew PhD; Wang, Kathryn PhD; Xia, Tai-he PhD; Call, Katherine M. PhD; Luo, Jun PhD; Wu, Xingyao MD, PhD; Zuccarino, Riccardo MD; Bacha, Alexa BS; Bai, Yunhong MD, PhD; Gutmann, Laurie MD; Feely, Shawna M.E. MS; Grider, Tiffany; Rossor, Alexander M. MD; Reilly, Mary M. MD; Shy, Michael E. MD; Svaren, John PhD
doi : 10.1212/WNL.0000000000012266
Volume 97(5), 3 August 2021, p e489-e500
To determine whether microRNAs (miRs) are elevated in the plasma of individuals with the inherited peripheral neuropathy Charcot-Marie-Tooth disease type 1A (CMT1A), miR profiling was employed to compare control and CMT1A plasma.
Spectrum of Clinical Features in X-Linked Myotubular Myopathy Carriers: An International Questionnaire Study
Reumers, Stacha F.I. MS*; Braun, Frederik MD*; Spillane, Jennifer E. PhD; Bohm, Johann PhD; Pennings, Maartje BS; Schouten, Meyke MD; van der Kooi, Anneke J. MD, PhD; Foley, A. Reghan MD; Bonnemann, Carsten G. MD; Kamsteeg, Erik-jan PhD; Erasmus, Corrie E. MD, PhD; Schara-Schmidt, Ulrike MD; Jungbluth, Heinz MD, PhD; Voermans, Nicol C. MD, PhD
doi : 10.1212/WNL.0000000000012236
Volume 97(5), 3 August 2021, p e501-e512
To characterize the spectrum of clinical features in a cohort of X-linked myotubular myopathy (XL-MTM) carriers, including prevalence, genetic features, clinical symptoms, and signs, as well as associated disease burden.
Selection Approach to Identify the Optimal Biomarker Using Quantitative Muscle MRI and Functional Assessments in Becker Muscular Dystrophy
van de Velde, Nienke M. MD; Hooijmans, Melissa T. PhD; Sardjoe Mishre, Aashley S.D. MSc; Keene, Kevin R. MD; Koeks, Zaida MD; Veeger, Thom T.J. MSc; Alleman, Iris; van Zwet, Erik W. PhD; Beenakker, Jan-Willem M. PhD; Verschuuren, Jan J.G.M. MD PhD; Kan, Hermien E. PhD; Niks, Erik H. MD PhD
doi : 10.1212/WNL.0000000000012233
Volume 97(5), 3 August 2021, p e513-e522
To identify the best quantitative fat-water MRI biomarker for disease progression of leg muscles in Becker muscular dystrophy (BMD) by applying a stepwise approach based on standardized response mean (SRM) over 24 months, correlations with baseline ambulatory tests, and reproducibility.
Clinical, Imaging, and Pathologic Characteristics of Patients With Right vs Left Hemisphere-Predominant Logopenic Progressive Aphasia
Buciuc, Marina MD; Duffy, Joseph R. PhD; Machulda, Mary M. PhD; Graff-Radford, Jonathan MD; Pham, Nha Trang Thu BS; Martin, Peter R. MS; Senjem, Matthew L. MS; Jack, Clifford R. Jr. MD; Ertekin-Taner, Nilufer MD, PhD; Dickson, Dennis W. MD; Lowe, Val J. MD; Whitwell, Jennifer L. PhD; Josephs, Keith Anthony MD, MST, MSc
doi : 10.1212/WNL.0000000000012322
Volume 97(5), 3 August 2021, p e523-e534
To assess and compare demographic, clinical, neuroimaging, and pathologic characteristics of a cohort of patients with right hemisphere-predominant vs left hemisphere-predominant logopenic progressive aphasia (LPA).
Chimeric Antigen Receptor T Cells for Glioblastoma: Current Concepts, Challenges, and Future Perspectives
Karschnia, Philipp MD, DSc*; Teske, Nico MD*; Thon, Niklas MD, DSc, MBA; Subklewe, Marion MD, DSc; Tonn, Joerg-Christian MD, DSc; Dietrich, Jorg MD, PhD; von Baumgarten, Louisa MD, DSc
doi : 10.1212/WNL.0000000000012193
Volume 97(5), 3 August 2021, p 218-230
Glioblastoma is the most common malignant primary brain tumor and is associated with a poor prognosis even after multimodal therapy. Chimeric antigen receptor (CAR) T cells have emerged as a promising therapeutic avenue in glioblastoma. CARs incorporate antigen-recognition moieties that endow autologous T cells with specificity against antigens expressed on glioblastoma (e.g., interleukin [IL]-13R[alpha]2, epidermal growth factor receptor variant III [EGFRvIII], and human epidermal growth factor receptor 2 [HER2]). Compelling antitumor effects of such therapy have been shown in murine glioblastoma models. In humans, 5 phase I/II studies on IL-13R[alpha]2-, EGFRvIII-, and HER2-directed CAR T cells for the treatment of glioblastoma have been published suggesting an acceptable safety profile. However, antitumor effects fell short of expectations in these initial clinical studies. Tumor heterogeneity, antigen loss, and the immunosuppressive tumor microenvironment are among the most important factors to limit the efficacy of CAR T-cell therapy in glioblastoma. Novel target antigens, modification of CAR T-cell design, the combination of CAR T-cell therapy with other therapeutic approaches, but also the use of CAR natural killer cells or CAR macrophages may optimize antitumor effects. Numerous clinical trials studying such approaches are ongoing, as well as several preclinical studies. With an increasing understanding of immune-escape mechanisms of glioblastoma and novel manufacturing techniques for CARs, CAR T cells may provide clinically relevant activity in glioblastoma. This review focuses on the use of CAR T cells in glioblastoma, but also introduces the basic structure, mechanisms of action, and relevant side effects of CAR T cells.
Cerebral Amyloid Angiopathy-Related Transient Focal Neurologic Episodes
Smith, Eric E. MD, MPH; Charidimou, Andreas MD, PhD; Ayata, Cenk MD; Werring, David J. MD; Greenberg, Steven M. MD, PhD
doi : 10.1212/WNL.0000000000012234
Volume 97(5), 3 August 2021, p 231-238
Transient focal neurologic episodes (TFNEs) are brief disturbances in motor, somatosensory, visual, or language functions that can occur in patients with cerebral amyloid angiopathy (CAA) and may be difficult to distinguish from TIAs or other transient neurologic syndromes. They herald a high rate of future lobar intracerebral hemorrhage, making it imperative to differentiate them from TIAs to avoid potentially dangerous use of antithrombotic drugs. Cortical spreading depression or depolarization triggered by acute or chronic superficial brain bleeding, a contributor to brain injury in other neurologic diseases, may be the underlying mechanism. This review discusses diagnosis, pathophysiology, and management of CAA-related TFNEs.
Quality Improvement in Neurology: 2020 Parkinson Disease Quality Measurement Set Update
Chou, Kelvin L. MD; Martello, Justin MD; Atem, Juliana MSN, APRN; Elrod, Matt DPT; Foster, Erin R. PhD, OTD, OTR/L; Freshwater, Karen PA-C; Gunzler, Steven A. MD; Kim, Hojoong MD; Mahajan, Abhimanyu MD, MHS; Sarva, Harini MD; Stebbins, Glenn T. PhD; Lee, Erin; Yang, Laurice MD, MHA
doi : 10.1212/WNL.0000000000012198
Volume 97(5), 3 August 2021, p 239-245
Intraventricular CSF Turbulence in Pediatric Communicating Hydrocephalus
Duy, Phan Q. BS; Kahle, Kristopher T. MD, PhD
doi : 10.1212/WNL.0000000000012237
Volume 97(5), 3 August 2021, p 246-247
Pearls & Oy-sters: Primary Pineal Melanoma With Leptomeningeal Carcinomatosis
Aaroe, Ashley E. MD; Glitza Oliva, Isabella C. MD, PhD; Al-Zubidi, Nagham MD; Nader, Marc-Elie MD; Kaya, Diana MD; Ferguson, Sherise D. MD; Wang, Chenyang MD, PhD; Fuller, Gregory MD, PhD; Harrison, Rebecca A. MD
doi : 10.1212/WNL.0000000000012121
Volume 97(5), 3 August 2021, p 248-250
Clinical Reasoning: A 47-Year-Old With Headache, Vertigo, and Double Vision
Frey, Jessica MD; Kramer, Joshua MD; Castellani, Rudolph MD; Sriwastava, Shitiz MD
doi : 10.1212/WNL.0000000000012138
Volume 97(5), 3 August 2021, p e535-e539
Teaching Video NeuroImage: Improvement in Motor Development After Start of Levodopa in Tyrosine Hydroxylase Deficiency
Janssen, Etienne MD; Oosterloo, Mayke MD, PhD; Rubio-Gozalbo, Estela MD, PhD; van Gassen, Koen PhD; Nicolai, Joost MD, PhD
doi : 10.1212/WNL.0000000000011757
Volume 97(5), 3 August 2021, p e540
Teaching Video NeuroImage: One Bedside Test, 2 Clinical Signs: One Vestibular, the Other Ocular Motor
Strupp, Michael MD; Frenzel, Claudia; Goldschagg, Nicolina MD; Halmagyi, G. Michael MD
doi : 10.1212/WNL.0000000000012080
Volume 97(5), 3 August 2021, p e541-e542
Editors' Note: Etiologic Uncertainties Regarding Neurologic Presentations Associated With COVID-19
Ganesh, Aravind MD, DPhil, FRCPC; Galetta, Steven MD,
doi : 10.1212/WNL.0000000000012358
Volume 97(5), 3 August 2021, p 251
Reader Response: Acute Necrotizing Encephalopathy With SARS-CoV-2 RNA Confirmed in Cerebrospinal Fluid
Frontera, Jennifer A.; Lewis, Ariane; Yaghi, Shadi; Bhimraj, Adarsh
doi : 10.1212/WNL.0000000000012357
Volume 97(5), 3 August 2021, p 252
Reader Response: Early Postmortem Brain MRI Findings in COVID-19 Non-survivors
Machado, Calixto
doi : 10.1212/WNL.0000000000012359
Volume 97(5), 3 August 2021, p 253
Reader Response: Early Postmortem Brain MRI Findings in COVID-19 Non-survivors
Demas, Alexis
doi : 10.1212/WNL.0000000000012360
Volume 97(5), 3 August 2021, p 253-254
Reader Response: Critical Illness-Associated Cerebral Microbleeds in COVID-19 Acute Respiratory Distress Syndrome
Edlow, Brian L.; Conklin, John; Huang, Susie Y.
doi : 10.1212/WNL.0000000000012364
Volume 97(5), 3 August 2021, p 254
