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Spotlight on the August 31 Issue
José G. Merino
doi : 10.1212/WNL.0000000000012508
vol. 97 no. 9 405-406
Differing Impact of Disease-Modifying Therapy on Relapse and Progression: Time to Consider Inflammation and Neuroprotection Separately?
Ruth Dobson, Amber Salter
doi : 10.1212/WNL.0000000000012356
vol. 97 no. 9 407-408
Not-So-Normal-Appearing White Matter
Leigh, Richard MD; Luby, Marie PhD
doi : 10.1212/WNL.0000000000012405
Volume 97(9), 31 August 2021, p 409-410
Effects of High- and Low-Efficacy Therapy in Secondary Progressive Multiple Sclerosis
Roos, Izanne MBChB, MMed (Neurology); Leray, Emmanuelle PhD; Casey, Romain PhD; Horakova, Dana MD, PhD; Havrdova, Eva MD, PhD; Izquierdo, Guillermo MD; Madueno, Sara Eichau MD; Patti, Francesco MD; Edan, Gilles MD, PhD; Debouverie, Marc MD, PhD; Pelletier, Jean MD, PhD; Ozakbas, Serkan MD; Amato, Maria Pia MD; Clavelou, Pierre MD, PhD; Grammond, Pierre MD; Boz, Cavit MD; Buzzard, Katherine MBBS, PhD; Skibina, Olga MBBS, PhD; Ciron, Jonathan MD; Gerlach, Oliver MD; Grand'Maison, Francois MD; Lechner-Scott, Jeannette MD, PhD; Malpas, Charles MPsych(CliNeuro), PhD; Butzkueven, Helmut MBBS, PhD; Vukusic, Sandra MD, PhD; Kalincik, Tomas MD, PhD, PGCertBiostat; and the MSBase and OFSEP Study Groups; and the MSBase and OFSEP Study Groups
doi : 10.1212/WNL.0000000000012354
Volume 97(9), 31 August 2021, p e869-e880
To compare the clinical effectiveness of high- and low-efficacy treatments in patients with recently active and inactive secondary progressive multiple sclerosis (SPMS) after accounting for therapeutic lag.
Association of Disease Severity and Socioeconomic Status in Black and White Americans With Multiple Sclerosis
Gray-Roncal, Karla MD; Fitzgerald, Kathryn C. ScD; Ryerson, Lana Zhovtis MD; Charvet, Leigh PhD; Cassard, Sandra D. ScD; Naismith, Robert MD; Ontaneda, Daniel MD, PhD; Mahajan, Kedar MD, PhD; Castro-Borrero, Wanda MD; Mowry, Ellen M. MD
doi : 10.1212/WNL.0000000000012362
Volume 97(9), 31 August 2021, p e881-e889
To compare clinical and imaging features of multiple sclerosis (MS) severity between Black Americans (BAs) and White Americans (WAs) and to evaluate the role of socioeconomic status.
Autologous Hematopoietic Stem Cell Transplantation in Active Multiple Sclerosis: A Real-world Case Series
Nicholas, Richard S. PhD*; Rhone, Elijah E. MD*; Mariottini, Alice MD; Silber, Eli MD; Malik, Omar MD, PhD; Singh-Curry, Victoria MD; Turner, Ben MD; Scalfari, Antonio MD, PhD; Ciccarelli, Olga MD, PhD; Sormani, Maria P. PhD; Olavarria, Eduardo MD; Mehra, Varun MD, PhD; Gabriel, Ian MD; Kazmi, Majid A. MD; Muraro, Paolo MD, PhD; on behalf of the London Group on Autologous Hematopoietic Stem Cell Transplantation for Multiple Sclerosis
doi : 10.1212/WNL.0000000000012449
Volume 97(9), 31 August 2021, p e890-e901
To examine outcomes in people with multiple sclerosis (PwMS) treated with autologous hematopoietic stem cell transplantation (AHSCT) in a real-world setting.
Cerebral Oxygen Metabolic Stress, Microstructural Injury, and Infarction in Adults With Sickle Cell Disease
Wang, Yan MD*; Fellah, Slim PhD*; Fields, Melanie E. MD, MSCI; Guilliams, Kristin P. MD, MSCI; Binkley, Michael M. PhD, MS; Eldeniz, Cihat PhD; Shimony, Joshua S. MD, PhD; Reis, Martin MD; Vo, Katie D. MD; Chen, Yasheng DSc; Lee, Jin-Moo MD, PhD; An, Hongyu DSc; Ford, Andria L. MD, MSCI
doi : 10.1212/WNL.0000000000012404
Volume 97(9), 31 August 2021, p e902-e912
To determine the patient- and tissue-based relationships between cerebral hemodynamic and oxygen metabolic stress, microstructural injury, and infarct location in adults with sickle cell disease (SCD).
Impact of Renal Impairment on Intensive Blood-Pressure-Lowering Therapy and Outcomes in Intracerebral Hemorrhage: Results From ATACH-2
Fukuda-Doi, Mayumi MD, MPH, PhD; Yamamoto, Haruko MD, PhD; Koga, Masatoshi MD, PhD; Doi, Yohei MD, PhD; Qureshi, Adnan I. MD; Yoshimura, Sohei MD, PhD; Miwa, Kaori MD, PhD; Ishigami, Akiko MD; Shiozawa, Masayuki MD; Omae, Katsuhiro PhD; Ihara, Masafumi MD, PhD; Toyoda, Kazunori MD, PhD
doi : 10.1212/WNL.0000000000012442
Volume 97(9), 31 August 2021, p e913-e921
The clinical effect of renal impairment on intracerebral hemorrhage (ICH) is unknown. This study sought to assess whether estimated glomerular filtration rate (eGFR) affects clinical outcomes or modifies the efficacy of intensive systolic blood pressure (BP) control (target, 110-139 mm Hg) against the standard (target, 140-179 mm Hg) among patients with ICH.
Cognitive Activity and Onset Age of Incident Alzheimer Disease Dementia
Wilson, Robert S. PhD; Wang, Tianhao PhD; Yu, Lei PhD; Grodstein, Francine PhD; Bennett, David A. MD; Boyle, Patricia A. PhD
doi : 10.1212/WNL.0000000000012388
Volume 97(9), 31 August 2021, p e922-e929
To test the hypothesis that higher level of cognitive activity predicts older age at dementia onset in Alzheimer disease (AD) dementia.
Diagnostic Value of the CSF [alpha]-Synuclein Real-Time Quaking-Induced Conversion Assay at the Prodromal MCI Stage of Dementia With Lewy Bodies
Rossi, Marcello MSc*; Baiardi, Simone MD, PhD*; Teunissen, Charlotte E. PhD; Quadalti, Corinne PhD; van de Beek, Marleen MSc; Mammana, Angela MSc; Stanzani-Maserati, Michelangelo MD, PhD; Van der Flier, Wiesje M. PhD; Sambati, Luisa MD, PhD; Zenesini, Corrado MSc; Caughey, Byron PhD; Capellari, Sabina MD; Lemstra, Afina W. MD, PhD; Parchi, Piero MD, PhD
doi : 10.1212/WNL.0000000000012438
Volume 97(9), 31 August 2021, p e930-e940
To investigate whether the CSF [alpha]-synuclein ([alpha]-syn) real-time quaking-induced conversion (RT-QuIC) assay accurately identifies patients with mild cognitive impairment (MCI) due to probable Lewy body (LB) disease.
Characterizing the Clinical Features and Atrophy Patterns of MAPT-Related Frontotemporal Dementia With Disease Progression Modeling
Young, Alexandra L. PhD; Bocchetta, Martina PhD; Russell, Lucy L. PhD; Convery, Rhian S. MSc; Peakman, Georgia MSc; Todd, Emily MRes; Cash, David M. PhD; Greaves, Caroline V. BSc; van Swieten, John MD; Jiskoot, Lize PhD; Seelaar, Harro MD, PhD; Moreno, Fermin MD; Sanchez-Valle, Raquel MD; Borroni, Barbara MD; Laforce, Robert Jr MD; Masellis, Mario MD, PhD; Tartaglia, Maria Carmela MD; Graff, Caroline MD; Galimberti, Daniela PhD; Rowe, James B. FRCP, PhD; Finger, Elizabeth MD; Synofzik, Matthis MD; Vandenberghe, Rik MD; de Mendonca, Alexandre MD, PhD; Tagliavini, Fabrizio MD; Santana, Isabel MD; Ducharme, Simon MD; Butler, Chris FRCP PhD; Gerhard, Alex MRCP, MD; Levin, Johannes MD; Danek, Adrian MD; Otto, Markus MD; Sorbi, Sandro; Williams, Steven C.R.; Alexander, Daniel C.; Rohrer, Jonathan D. PhD, FRCP; on behalf of the Genetic FTD Initiative (GENFI); on behalf of the Genetic FTD Initiative (GENFI); Rossor, Martin N. MD, FRCP; Fox, Nick C. MD, FRCP; Warren, Jason D. PhD, FRACP; Woollacott, Ione MRCP; Shafei, Rachelle MRCP; Heller, Carolin MSc; Swift, Imogen J MSc; Moore, Katrina PhD; Guerreiro, Rita PhD; Bras, Jose PhD; Thomas, David L. PhD; Nicholas, Jennifer PhD; Mead, Simon PhD; Meeter, Lieke MD; Panman, Jessica MSc; Papma, Janne M. PhD; Poos, Jackie MSc; van Minkelen, Rick PhD; Pijnenburg, Yolande MD, PhD; Barandiaran, Myriam PhD; Indakoetxea, Begona MD; Gabilondo, Alazne MD; Tainta, Mikel MD; de Arriba, Maria BSc; Gorostidi, Ana PhD; Zulaica, Miren BSc; Villanua, Jorge MD, PhD; Diaz, Zigor; Borrego-Ecija, Sergi MD; Olives, Jaume MSc; Llado, Albert PhD; Balasa, Mircea PhD; Antonell, Anna PhD; Bargallo, Nuria PhD; Premi, Enrico MD; Cosseddu, Maura MPsych; Gazzina, Stefano MD; Padovani, Alessandro MD, PhD; Gasparotti, Roberto MD; Archetti, Silvana MBiolSci; Black, Sandra MD; Mitchell, Sara MD; Rogaeva, Ekaterina PhD; Freedman, Morris MD; Keren, Ron MD; Tang-Wai, David MD; Oijerstedt, Linn MD; Andersson, Christin PhD; Jelic, Vesna MD; Thonberg, Hakan MD; Arighi, Andrea MD; Fenoglio, Chiara PhD; Scarpini, Elio MD; Fumagalli, Giorgio MD; Cope, Thomas MRCP; Timberlake, Carolyn BSc; Rittman, Timothy MRCP; Shoesmith, Christen MD; Bartha, Robart PhD; Rademakers, Rosa PhD; Wilke, Carlo MD; Karnath, Hans Otto MD; Bender, Benjamin MD; Bruffaerts, Rose MD, PhD; Van Damme, Philip MD, PhD; Vandenbulcke, Mathieu MD, PhD; Ferreira, Catarina B. MSc; Miltenberger, Gabriel PhD; Maruta, Carolina MPsych, PhD; Verdelho, Ana MD, PhD; Afonso, Sonia BSc; Taipa, Ricardo MD, PhD; Caroppo, Paola MD, PhD; Di Fede, Giuseppe MD, PhD; Giaccone, Giorgio MD; Prioni, Sara PsyD; Redaelli, Veronica MD; Rossi, Giacomina MSc; Tiraboschi, Pietro MD; Duro, Diana MPsych; Almeida, Maria Rosario PhD; Branco, Miguel Castelo MD, PhD; Leitao, Maria Joao BSc; Pereira, Miguel Tabuas MD; Santiago, Beatriz MD; Gauthier, Serge MD; Neto, Pedro Rosa MD, PhD; Veldsman, Michele PhD; Thompson, Paul; Prix, Catharina MD; Hoegen, Tobias MD; Mag.rer.nat, Elisabeth Wlasich; Loosli, Sandra MD; Schonecker, Sonja MD; Dr.hum.bio, Elisa Semler; Psych, Dipl.; Anderl-Straub, Sarah; Psych, Dipl.; Nacmias, Benedetta; Ferrari, Camilla; Polito, Cristina; Lombardi, Gemma; Bessi, Valentina
doi : 10.1212/WNL.0000000000012410
Volume 97(9), 31 August 2021, p e941-e952
Mutations in the MAPT gene cause frontotemporal dementia (FTD). Most previous studies investigating the neuroanatomical signature of MAPT mutations have grouped all different mutations together and shown an association with focal atrophy of the temporal lobe. The variability in atrophy patterns between each particular MAPT mutation is less well-characterized. We aimed to investigate whether there were distinct groups of MAPT mutation carriers based on their neuroanatomical signature.
Feasibility and Biological Activity of a Ketogenic/Intermittent-Fasting Diet in Patients With Glioma
Schreck, Karisa C. MD, PhD; Hsu, Fang-Chi PhD; Berrington, Adam PhD; Henry-Barron, Bobbie RD; Vizthum, Diane MS, RD; Blair, Lindsay RN; Kossoff, Eric H. MD; Easter, Linda MS, RD; Whitlow, Christopher T. MD, PhD, MHA; Barker, Peter B. PhD; Cervenka, Mackenzie C. MD; Blakeley, Jaishri O. MD; Strowd, Roy E. MD, MS, MEd, FAAN
doi : 10.1212/WNL.0000000000012386
Volume 97(9), 31 August 2021, p e953-e963
To examine the feasibility, safety, systemic biological activity, and cerebral activity of a ketogenic dietary intervention in patients with glioma.
Gerstmann Syndrome Deconstructed by Cortical Stimulation
Vaddiparti, Aparna MD; McGrath, Hari; Benjamin, Christopher F.A. PhD; Sivaraju, Adithya MD; Spencer, Dennis D. MD; Hirsch, Lawrence J. MD; Damisah, Eyiyemisi MD*; Quraishi, Imran H. MD, PhD*
doi : 10.1212/WNL.0000000000012441
Volume 97(9), 31 August 2021, p 420-422
Deciphering Neurodegenerative Diseases Using Long-Read Sequencing
Su, Yun MD*; Fan, Liyuan MD*; Shi, Changhe MD, PhD*; Wang, Tai MD; Zheng, Huimin MD; Luo, Haiyang MD, PhD; Zhang, Shuo MD, PhD; Hu, Zhengwei MD, PhD; Fan, Yu MD, PhD; Dong, Yali MD; Yang, Jing MD, PhD; Mao, Chengyuan MD, PhD; Xu, Yuming MD, PhD
doi : 10.1212/WNL.0000000000012466
Volume 97(9), 31 August 2021, p 423-433
Neurodegenerative diseases exhibit chronic progressive lesions in the central and peripheral nervous systems with unclear causes. The search for pathogenic mutations in human neurodegenerative diseases has benefited from massively parallel short-read sequencers. However, genomic regions, including repetitive elements, especially with high/low GC content, are far beyond the capability of conventional approaches. Recently, long-read single-molecule DNA sequencing technologies have emerged and enabled researchers to study genomes, transcriptomes, and metagenomes at unprecedented resolutions. The identification of novel mutations in unresolved neurodegenerative disorders, the characterization of causative repeat expansions, and the direct detection of epigenetic modifications on naive DNA by virtue of long-read sequencers will further expand our understanding of neurodegenerative diseases. In this article, we review and compare 2 prevailing long-read sequencing technologies, Pacific Biosciences and Oxford Nanopore Technologies, and discuss their applications in neurodegenerative diseases.
Randomized Study of Bedside vs Hallway Rounding: Neurology Rounding Study
Solomon, Jacqueline M. MD; Bhattacharyya, Shamik MD; Ali, Ahya S. MBBS; Cleary, Liam BA; Dibari, Sarah BSN; Boxer, Robert MD, PhD; Healy, Brian C. PhD; Milligan, Tracey A. MD
doi : 10.1212/WNL.0000000000012407
Volume 97(9), 31 August 2021, p 434-442
Over the last century, attending rounds have shifted away from the bedside. Despite evidence for greater patient satisfaction rates and improved nursing perception of teamwork with bedside presentations, residents and attending physicians are apprehensive of the bedside approach. There is lack of data to guide rounding practices within neurology, and therefore, optimal rounding methods remain unclear. The objective of this study was to compare bedside rounding with hallway rounding on an academic neurology inpatient service and assess efficiency, trainee education, and satisfaction among patients and staff. We conducted a single-center prospective randomized study of bedside vs hallway rounding on new inpatient neurology admissions over 1-week blocks. The bedside team presented patients at the bedside, whereas the hallway team presented patients outside of the patient's room. We evaluated the 2 approaches with time-motion analysis, which investigated the rounding style's effect on composition and timing of rounds (primary outcome) and surveys of patients, nurses, residents, and attending physicians on both teams (secondary outcomes). The mean rounding time per newly admitted patient in the bedside group (n = 38 patients) and hallway group (n = 41 patients) was 23 minutes and 23.2 minutes, respectively (p = 0.93). The bedside group spent on average 56.4% of patient rounding time in the patient's room, whereas the hallway group spent 39.5% of rounding time in the patient's room (p = 0.036). Residents perceived hallway rounding to be more efficient and associated it with a superior educational experience and more effective data review. Nurses had improved perception of their participation in bedside rounds. Although patients' views of bedside and hallway rounds were similar, patients who had experienced bedside rounds preferred it. In conclusion, bedside rounding was perceived less favorably by most residents but was as efficient as hallway rounding. Although bedside rounding limited the use of technology for data review, it promoted nursing participation and resulted in more time spent with the patient.
Treatment of Painless Legs and Moving Toes Syndrome Associated With Multiple System Atrophy With Levodopa
Araki, Takeya MD; Mukai, Tatsuya MD; Araki, Takehisa MD, PhD
doi : 10.1212/WNL.0000000000012303
Volume 97(9), 31 August 2021, p 443-444
Resident & Fellow Rounds: August 2021
Strowd, Roy E. III MD, MEd, MS, FAAN; Editor; Aamodt, Whitley MD, MPH; Deputy Editor
doi : 10.1212/WNL.0000000000012509
Volume 97(9), 31 August 2021, p 445-448
Clinical Reasoning: A 42-Year-Old Woman With Mysterious Monocytic Meningitis
Nothem, Meghan E. DO; Lee, Ryan M. MD, PhD; Katz, Jonathan M. BS; Bergl, Paul A. MD; Obeidat, Ahmed Z. MD, PhD
doi : 10.1212/WNL.0000000000012155
Volume 97(9), 31 August 2021, p 449-454
Pearls & Oy-sters: Angioplasty and Stenting as New Treatment Method for Cough Headache With Stenotic Internal Jugular Vein: Case Report With 12-Month Follow-up
Cao, Xiangyu MD*; Li, Baomin MD*; Su, Hui MD; Wang, Rongfei MD; Wang, Jun MD; Tian, Chenglin MD; Du, Zhihua MD; Liu, Xinfeng MD; Zhang, Yunxia MD; Chen, Xing PhD; Dong, Zhao MD; Yu, Shengyuan MD
doi : 10.1212/WNL.0000000000012152
Volume 97(9), 31 August 2021, p e964-e967
Teaching NeuroImage: Symmetric Deep Cerebellar White Matter T2 and Susceptibility-Weighted Imaging Hypointense Lesions in a Case of Cerebrotendinous Xanthomatosis
Zhang, Yue MD, PhD*; Sun, Yi-Min MD, PhD*; Li, Haiqing MD, PhD
doi : 10.1212/WNL.0000000000012154
Volume 97(9), 31 August 2021, p e968-e969
Teaching Video NeuroImage: "Weighing" in on an Unusual Tremor
Villa-Lopez, Marta MD; Oh, Eungseok MD, PhD; Chen, Robert MD; Lang, Anthony E. MD, FRCPC; Masellis, Mario MSc, MD, PhD, FRCPC*; Hopyan, Julia J. MD, FRCPC*
doi : 10.1212/WNL.0000000000012141
Volume 97(9), 31 August 2021, p e970-e971
Comparing the Effectiveness of Therapies in People With Secondary Progressive Multiple Sclerosis
Rice, Jessica MD; Xiang, Xinran Maria MD
doi : 10.1212/WNL.0000000000012510
Volume 97(9), 31 August 2021, p e972-e974
Editors' Note: Scan-Negative Cauda Equina Syndrome: A Prospective Cohort Study
Siegler, James E. III MD; Galetta, Steven MD
doi : 10.1212/WNL.0000000000012501
Volume 97(9), 31 August 2021, p 455
Reader Response: Scan-Negative Cauda Equina Syndrome: A Prospective Cohort Study
Amelot, Aymeric; Planty-Bonjour, Alexia; Terrier, Louis-Marie
doi : 10.1212/WNL.0000000000012502
Volume 97(9), 31 August 2021, p 455-456
Author Response: Scan-Negative Cauda Equina Syndrome: A Prospective Cohort Study
Hoeritzauer, Ingrid; Carson, Alan; Statham, Patrick; Demetriades, Andreas; Stone, Jon
doi : 10.1212/WNL.0000000000012503
Volume 97(9), 31 August 2021, p 456-457
Reader Response: Scan-Negative Cauda Equina Syndrome: A Prospective Cohort Study
Beucler, Nathan
doi : 10.1212/WNL.0000000000012504
Volume 97(9), 31 August 2021, p 457
Editors' Note: Association of Age at Onset and First Symptoms With Disease Progression in Patients With Metachromatic Leukodystrophy
Siegler, James E. III MD; Galetta, Steven MD
doi : 10.1212/WNL.0000000000012505
Volume 97(9), 31 August 2021, p 457-458
Reader Response: Association of Age at Onset and First Symptoms With Disease Progression in Patients With Metachromatic Leukodystrophy
Michell-Robinson, Mackenzie A.; Lepine, Sarah
doi : 10.1212/WNL.0000000000012506
Volume 97(9), 31 August 2021, p 458
Author Response: Association of Age at Onset and First Symptoms With Disease Progression in Patients With Metachromatic Leukodystrophy
Groeschel, Samuel; Krageloh-Mann, Ingeborg
doi : 10.1212/WNL.0000000000012507
Volume 97(9), 31 August 2021, p 459
Opinion and Special Articles: Remote Evaluation of Acute Vertigo: Strategies and Technological Considerations
doi : 10.1212/WNL.0000000000012239
Volume 97(9), 31 August 2021, p 459
