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Spotlight on the November 1 Issue.
Merino, Jose G. MD, MPhil; Editor-in-Chief, Neurology
doi : 10.1212/WNL.0000000000201281
Volume 99(18) pgs. 775-819,e1957-e2080 November 1, 2022
Lesion-Induced Dystonia: Lessons Learned From Brain Network Studies.
Chen, Chiung Chu MD, PhD
Headaches in Veterans: Different or the Same?.
Finkel, Alan G. MD, FAAN
Caring for Our Sickest Patients: The Expanding Role of the Pediatric Neurohospitalist.
Benedetti, Giulia M. MD; Amlie-Lefond, Catherine MD
Clinical and Structural Findings in Patients With Lesion-Induced Dystonia: Descriptive and Quantitative Analysis of Published Cases.
Corp, Daniel T. PhD; Greenwood, Christopher J. PhD; Morrison-Ham, Jordan BS; Pullinen, Jaakko MD; McDowall, Georgia M. BS; Younger, Ellen F. P. BS; Jinnah, Hyder A. MD; Fox, Michael D. MD, PhD; Joutsa, Juho MD, PhD
doi : 10.1212/WNL.0000000000201042
Brain lesions are a well-recognized etiology of dystonia. These cases are especially valuable because they offer causal insight into the neuroanatomical substrates of dystonia. To date, knowledge of lesion-induced dystonia comes mainly from isolated case reports or small case series, restricting broader description and analysis.
Headache, Opiate Use, and Prescribing Trends in Women With Idiopathic Intracranial Hypertension: A Population-Based Matched Cohort Study.
Adderley, Nicola Jaime MSci, MA, MPhil, PhD, MPH; Subramanian, Anuradhaa MSc; Perrins, Mary MBChB; Nirantharakumar, Krishnarajah MBBS, MPH, MFPH, MRCP(UK), MD; Mollan, Susan P. MBChB; Sinclair, Alexandra Jean PhD
doi : 10.1212/WNL.0000000000201064
Physician prescribing habits for opiates and headache therapies have not been previously evaluated in a large, matched cohort study in idiopathic intracranial hypertension (IIH). Our objective was to evaluate opiate and headache medication prescribing habits in women with IIH compared with matched women with migraine and population controls.
Frequency, Demographics, Comorbidities, and Health Care Utilization by Veterans With Migraine: A VA Nationwide Cohort Study.
Seng, Elizabeth K. PhD; Fenton, Brenda T. PhD, MSc; Wang, Kaicheng MD MPH; Lipton, Richard B. MD; Ney, John MD, MPH; Damush, Teresa PhD; Grinberg, Amy S. PhD; Skanderson, Melissa MS; Sico, Jason J. MD, MHS, FAHA
doi : 10.1212/WNL.0000000000200888
To describe the relative frequency, demographics, comorbidities, and health care utilization of veterans who receive migraine care at the Veteran's Health Administration (VHA) and to evaluate differences by gender. Methods: This study extracted data from VHA administrative sources. Veterans diagnosed with migraine by a health care provider between fiscal year 2008 and 2019 were included.
Characteristics and Gender Differences of Headache in the Veterans Health Administration: A National Cohort Study, Fiscal Year 2008-2019.
Sico, Jason Jonathon MD, MHS, FAHA; Seng, Elizabeth K. PhD; Wang, Kaicheng MD, MPH; Skanderson, Melissa MS; Schindler, Emmanuelle A.D. MD, PhD; Ney, John P. MD, MPH; Lorenze, Nancy DNS, MSN; Kimber, Addison BA; Lindsey, Hayley BA; Grinberg, Amy S. PhD; Kuruvilla, Deena MD; Higgins, Donald S. MD; Graham, Glenn MD, PhD; Sandbrink, Friedhelm MD; Scholten, Joel MD; Shapiro, Robert E. MD, PhD; Lipton, Richard B. MD; Fenton, Brenda T. PhD MSc
doi : 10.1212/WNL.0000000000200905
To determine gender differences in headache types diagnosed, sociodemographic characteristics, military campaign and exposures, and health care utilization among US veterans in the Veterans Health Administration (VHA). Methods: This study used a retrospective cohort design to examine VHA electronic health record (EHR) data. This cohort includes veterans who had at least 1 visit for any headache between fiscal years 2008 and 2019.
Prevalence and Risk Factors for Pharmacoresistance in Children With Focal Cortical Dysplasia-Related Epilepsy.
Cohen, Nathan T. MD; Chang, Phat MPH; You, Xiaozhen PhD; Zhang, Anqing PhD; Havens, Kathryn A. PA-C; Oluigbo, Chima O. MD; Whitehead, Matthew T. MD; Gholipour, Taha MD; Gaillard, William D. MD
doi : 10.1212/WNL.0000000000201033
Focal cortical dysplasia (FCD) is the most common cause of surgically remediable epilepsy in children. Little is known about the risk factors for the timing and development of pharmacoresistance in this population. This study sought to evaluate the prevalence and risk factors for pharmacoresistance in pediatric FCD-related epilepsy. Methods: In this retrospective single-center cohort design, patients were identified from search of centralized radiology report database and a central epilepsy surgical database.
Herpes Viruses in the Baltimore Longitudinal Study of Aging: Associations With Brain Volumes, Cognitive Performance, and Plasma Biomarkers.
Duggan, Michael R. PhD; Peng, Zhongsheng MD, PhD; An, Yang MS; Kitner Triolo, Melissa H. PhD; Shafer, Andrea T. PhD; Davatzikos, Christos PhD; Erus, Guray PhD; Karikkineth, Ajoy MD; Lewis, Alexandria BS; Moghekar, Abhay MBBS, MD; Walker, Keenan A. PhD
doi : 10.1212/WNL.0000000000201036
Although an infectious etiology of Alzheimer disease (AD) has received renewed attention with a particular focus on herpes viruses, the longitudinal effects of symptomatic herpes virus (sHHV) infection on brain structure and cognition remain poorly understood, as does the effect of sHHV on AD/neurodegeneration biomarkers.
The State of Inpatient Child Neurology: A Survey of North American Academic Programs.
Nash, Kendall B. MD *; Palaganas, Jamie MD *; Abend, Nicholas S. MD, MSCE; Hutchinson, Melissa L. MD; Messer, Ricka MD, PhD; Moharir, Mahendranath MD; Piantino, Juan MD; Press, Craig A. MD, PhD; Wells, Elizabeth MD; for the Pediatric Neurohospitalist Work Group
doi : 10.1212/WNL.0000000000201101
Inpatient child neurology programs provide essential services for children. We sought to understand the current structure and challenges of inpatient pediatric neurologic care delivery in academic programs in North America.
Perception of Fragmented Letters by Patients With Pathologically Confirmed Dementia With Lewy Bodies or Alzheimer Disease.
Salmon, David P. PhD; Smirnov, Denis S. PhD; Coughlin, David G. MD; Hamilton, Joanne M. PhD; Landy, Kelly M. PhD; Filoteo, J. Vincent PhD; Hiniker, Annie MD, PhD; Hansen, Lawrence A. MD; Galasko, Douglas MD
doi : 10.1212/WNL.0000000000201068
Patients with dementia with Lewy bodies perform worse than those with Alzheimer disease (AD) on tests of visual perception, but the clinical utility of these tests remains unknown because studies often had clinically diagnosed groups that may inadvertently cross-contaminate Lewy body disease (LBD) with pure AD pathology, used experimental tests not easily adaptable for clinical use, and had no way to examine relationships between the severity of LBD pathology and degree of cognitive impairment.
Enhanced Imaging and Language Assessments for Primary Progressive Aphasia.
Ruch, Kristina BA; Stockbridge, Melissa Dawn PhD; Walker, Alexandra MA; Vitti, Emilia MS; Shea, Jennifer BA; Sheppard, Shannon PhD; Pacl, Alex MA; Kim, Hana PhD; Faria, Andreia Vasconcellos MD; Hillis, Argye Elizabeth MD, MA
doi : 10.1212/WNL.0000000000201040
It is widely agreed that primary progressive aphasia (PPA) is a clinical syndrome with at least 3 distinct variants that differ in phenotype, areas of atrophy, and most common underlying neurodegenerative disease. The distinction between logopenic variant PPA (lvPPA) and other variants is important for prognosis and medical management. However, differentiating logopenic from nonfluent agrammatic variant can be difficult.
Behavioral and Cognitive Phenotypes of Patients With Amyotrophic Lateral Sclerosis Carrying SOD1 Variants.
Dalla Bella, Eleonora MD; Bersano, Enrica MD; Bruzzone, Maria Grazia MD; Gellera, Cinzia PhD; Pensato, Viviana PhD; Lauria, Giuseppe MD; Consonni, Monica PhD
doi : 10.1212/WNL.0000000000201044
SOD1 variants in patients with amyotrophic lateral sclerosis (ALS) have been associated with peculiar clinical features and disease progression but rarely with cognitive and behavioral impairment.
Association of Blood Biomarkers of Inflammation With Penumbra Consumption After Mechanical Thrombectomy in Patients With Acute Ischemic Stroke.
Mechtouff, Laura MD, PhD *; Debs, Noelie PhD *; Frindel, Carole PhD; Bani-Sadr, Alexandre MD; Bochaton, Thomas MD, PhD; Paccalet, Alexandre PhD; Crola Da Silva, Claire PhD; Buisson, Marielle PharmD; Amaz, Camille Msc; Berthezene, Yves MD, PhD; Eker, Omer Faruk MD, PhD; Bouin, Morgane; de Bourguignon, Charles Msc; Mewton, Nathan MD, PhD; Ovize, Michel MD, PhD; Bidaux, Gabriel PhD; Nighoghossian, Norbert MD *; Cho, Tae-Hee MD *
doi : 10.1212/WNL.0000000000201038
The objective of this study was to assess the relationship between blood biomarkers of inflammation and lesion growth within the penumbra in acute ischemic stroke (AIS) patients treated with mechanical thrombectomy (MT). Methods: The HIBISCUS-STROKE cohort enrolled patients admitted in the Lyon Stroke Center for an anterior circulation AIS treated with MT after brain MRI assessment.
Early Readmissions After Hospitalization for Posterior Reversible Encephalopathy Syndrome.
Garg, Aayushi MD; Elmashala, Amjad MD; Roeder, Hannah MD
doi : 10.1212/WNL.0000000000201086
To evaluate the frequency, etiologies, and risk factors for 90-day readmissions after hospitalization for posterior reversible encephalopathy syndrome (PRES). Methods: Data were obtained from the Nationwide Readmissions Database 2016-2018. Patients with a primary diagnosis of PRES, survival to discharge, and known discharge disposition were included. The primary outcome was nonelective readmission within 90 days of discharge.
Frequency of New or Enlarging Lesions on MRI Outside of Clinical Attacks in Patients With MOG-Antibody-Associated Disease.
Syc-Mazurek, Stephanie B. MD, PhD; Chen, John J. MD, PhD; Morris, Pearse MBBCh; Sechi, Elia MD; Mandrekar, Jaywant PhD; Tillema, Jan-Mendelt MD; Lopez-Chiriboga, A. Sebastian MD; Lucchinetti, Claudia Francesca MD; Zalewski, Nicholas MD; Cacciaguerra, Laura MD; Buciuc, Marina MD; Krecke, Karl N. MD; Messina, Steven Anthony MD; Bhatti, M. Tariq MD; Pittock, Sean J. MD; Flanagan, Eoin P. MBBCh
doi : 10.1212/WNL.0000000000201263
To determine the frequency of new or enlarging T2-hyperintense or enhancing lesions outside of clinical attacks in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) vs multiple sclerosis (MS) and aquaporin-4 antibody positive neuromyelitis optica spectrum disorder (AQP4+NMOSD)
Clinical Reasoning: A 32-Year-Old Woman With Tunnel Vision and Back Pain.
Primiani, Christopher T. MD; Hale, David E. MD; Shah, Manali MD; Green, Kemar E. DO
doi : 10.1212/WNL.0000000000201227
AB The incidence of new onset visual disturbances in emergency departments across the country is frequent. A detailed history of events and thoughtful physical examination may produce a diagnosis; however, atypical cases may require further diagnostic testing to explain symptoms. We present a case of presumed increased intracranial pressure with atypical findings on diagnostic testing, which allowed our team to explore a broader differential diagnosis.
Clinical Reasoning: A 12-Month-Old Male Child With Staring Episodes, Ataxia, and Right-sided Weakness.
Brosius, Stephanie N. MD, PhD; Otto, William MD, MSCE; Waldman, Amy MD, MSCE; Russo, Michael MD; McGuire, Jennifer MD, MSCE
doi : 10.1212/WNL.0000000000201233
AB Baylisascaris procyonis, or raccoon roundworm, is a rare cause of eosinophilic meningoencephalitis with historically poor clinical outcomes. Symptoms of neural larval migrans begin approximately 2-4 weeks after ingestion with fatigue, nausea, fever, and lethargy and then rapidly progress to weakness, incoordination, ataxia, seizures, altered mental status, and finally coma.
Pearls & Oy-sters: Genetic Epilepsy: An Important Cause of Neonatal Seizures in Infants With Complex Congenital Heart Disease.
Kazazian, Vanna MN; Selvanathan, Thiviya MD; Chau, Vann MD; Tam, Emily W.Y. MDCM, MAS; Miller, Steven P. MDCM, MAS
doi : 10.1212/WNL.0000000000201229
AB Genetic epilepsies, such as KCNQ2 gene variants, although uncommon, are potential causes of neonatal seizures in infants with complex congenital heart disease (CHD). KCNQ2-related seizures commonly present as tonic posturing with autonomic changes and a distinctive amplitude-integrated EEG (aEEG) pattern with increase in amplitude, immediately followed by background suppression.
Teaching NeuroImage: Selectively Bright Inferior Cerebellum in Christianson Syndrome.
Alves, Cesar Augusto P.F. MD, PhD; Clifford, Simon M. MD; McKeown Ruggiero, Sarah CGC; Helbig, Ingo MD; Chadehumbe, Madeline MD; Shekdar, Karuna MD
Editors' Note: Identification and Characterization of Pure Sleep Epilepsy in a Cohort of Patients With a First Seizure.
Lewis, Ariane MD; Galetta, Steven MD
Reader Response: Identification and Characterization of Pure Sleep Epilepsy in a Cohort of Patients With a First Seizure.
Coebergh, Jan A.
Author Response: Identification and Characterization of Pure Sleep Epilepsy in a Cohort of Patients With a First Seizure.
Pang, Elaine W.
Editors' Note: The Economic Burden of Multiple Sclerosis in the United States: Estimate of Direct and Indirect Costs.
Lewis, Ariane MD; Galetta, Steven MD
Reader Response: The Economic Burden of Multiple Sclerosis in the United States: Estimate of Direct and Indirect Costs.
Vargas, Wendy S.
Author Response: The Economic Burden of Multiple Sclerosis in the United States: Estimate of Direct and Indirect Costs.
Bebo, Bruce F.
