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August 2, 2022; 99 (5)

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In Focus

  • Spotlight on the August 2 Issue
    José G. Merino
    • Full Text
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Editorials

  • What's in a Domain? The Role of NOTCH3 EGFr Domains in CADASIL Disease Severity
    Remco Hack, Julie Rutten, Saskia A.J. Lesnik Oberstein
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  • Advances and Remaining Challenges in Distinguishing Variants of Primary Progressive Aphasia
    Argye Elizabeth Hillis, Kristina Ruch
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  • Greater Efficacy of Avalglucosidase vs Alglucosidase Alfa in Adult Pompe Disease?The Jury Is Still Out
    Marianne de Visser, Zohar Argov
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Research Articles

  • Open Access
    Association of NOTCH3 Variant Position With Stroke Onset and Other Clinical Features Among Patients With CADASIL
    Bernard P.H. Cho, Amy A. Jolly, Stefania Nannoni, Daniel Tozer, Steven Bell, Hugh S. Markus
    • Abstract
    • Full Text
    • Full Text (PDF)
  • Open Access
    Prevalence and Significance of the Vessel-Cluster Sign on Susceptibility-Weighted Imaging in Patients With Severe Small Vessel Disease
    Salvatore Rudilosso, Ernest Chui, Michael S. Stringer, Michael Thrippleton, Francesca Chappell, Gordon W. Blair, Daniela Jaime Garcia, Fergus Doubal, Iona Hamilton, Anna Kopczak, Michael Ingrisch, Danielle Kerkhofs, Walter H. Backes, Julie Staals, Robert van Oostenbrugge, Marco Duering, Martin Dichgans, Joanna M. Wardlaw, on behalf of the SVDs@Target Investigators
    • Abstract
    • Full Text
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    CME Course
  • Open Access
    Prevalence and Predictors of Vascular Cognitive Impairment in Patients With CADASIL
    Amy A. Jolly, Stefania Nannoni, Hayley Edwards, Robin G. Morris, Hugh S. Markus
    • Abstract
    • Full Text
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  • Open Access
    Association of APOE-Independent Alzheimer Disease Polygenic Risk Score With Brain Amyloid Deposition in Asymptomatic Older Adults
    Laura Xicota, Beata Gyorgy, Benjamin Grenier-Boley, Alexandre Lecoeur, Gaëlle Fontaine, Fabrice Danjou, Jorge Samper Gonzalez, Olivier Colliot, Philippe Amouyel, Garance Martin, Marcel Levy, Nicolas Villain, Marie-Odile Habert, Bruno Dubois, Jean-Charles Lambert, Marie-Claude Potier, on behalf of the INSIGHT pre-AD study group and for the Alzheimer's Disease Neuroimaging Initiative
    • Abstract
    • Full Text
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  • Open Access
    β-Amyloid–Dependent and –Independent Genetic Pathways Regulating CSF Tau Biomarkers in Alzheimer Disease
    Atul Kumar, Shorena Janelidze, Erik Stomrud, Sebastian Palmqvist, Oskar Hansson, Niklas Mattsson-Carlgren, for the Alzheimer's Disease Neuroimaging Initiative
    • Abstract
    • Full Text
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  • Sensitivity of the Social Behavior Observer Checklist to Early Symptoms of Patients With Frontotemporal Dementia
    Gianina Toller, Yann Cobigo, Peter A. Ljubenkov, Brian S. Appleby, Bradford C. Dickerson, Kimiko Domoto-Reilly, Jamie C. Fong, Leah K. Forsberg, Ralitza H. Gavrilova, Nupur Ghoshal, Hilary W. Heuer, David S. Knopman, John Kornak, Maria I. Lapid, Irene Litvan, Diane E. Lucente, Ian R. Mackenzie, Scott M. McGinnis, Bruce L. Miller, Otto Pedraza, Julio C. Rojas, Adam M. Staffaroni, Bonnie Wong, Zbigniew K. Wszolek, Brad F. Boeve, Adam L. Boxer, Howard J. Rosen, Katherine P. Rankin, and the ALLFTD research consortium
    • Abstract
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  • Automated Detection of Speech Timing Alterations in Autopsy-Confirmed Nonfluent/Agrammatic Variant Primary Progressive Aphasia
    Adolfo M. García, Ariane E. Welch, Maria Luisa Mandelli, Maya L. Henry, Sladjana Lukic, María José Torres Prioris, Jessica Deleon, Buddhika M. Ratnasiri, Diego L. Lorca-Puls, Bruce L. Miller, William Seeley, Adam P. Vogel, Maria Luisa Gorno-Tempini
    • Abstract
    • Full Text
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  • Presymptomatic Lesion in Childhood Cerebral AdrenoleukodystrophyTiming and Treatment
    Eric James Mallack, Keith P. Van Haren, Anna Torrey, Stephanie van de Stadt, Marc Engelen, Gerald V. Raymond, Ali Fatemi, Florian S. Eichler
    • Abstract
    • Full Text
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  • Sensory Phenotypes for Balance Dysfunction After Mild Traumatic Brain Injury
    Peter C. Fino, Leland E. Dibble, Elisabeth A. Wilde, Nora F. Fino, Paula Johnson, Melissa M. Cortez, Colby R. Hansen, Susanne M. van der Veen, Karen M. Skop, J. Kent Werner, David F. Tate, Harvey S. Levin, Mary Jo V. Pugh, William C. Walker
    • Abstract
    • Full Text
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    CME Course
  • Open Access
    Long-term Safety and Efficacy of Avalglucosidase Alfa in Patients With Late-Onset Pompe Disease
    Mazen M. Dimachkie, Richard J. Barohn, Barry Byrne, Ozlem Goker-Alpan, Priya S. Kishnani, Shafeeq Ladha, Pascal Laforêt, Karl Eugen Mengel, Loren D.M. Peña, Sabrina Sacconi, Volker Straub, Jaya Trivedi, Philip Van Damme, Ans T. van der Ploeg, John Vissing, Peter Young, Kristina An Haack, Meredith Foster, Jane M. Gilbert, Patrick Miossec, Olivier Vitse, Tianyue Zhou, Benedikt Schoser, on behalf of the NEO-EXT investigators
    • Abstract
    • Full Text
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Reviews

  • α-Synuclein Seed Amplification Assays for Diagnosing SynucleinopathiesThe Way Forward
    Giovanni Bellomo, Chiara Maria Giulia De Luca, Federico Paolini Paoletti, Lorenzo Gaetani, Fabio Moda, Lucilla Parnetti
    • Abstract
    • Full Text
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Video NeuroImages

  • Early-Onset Dystonia, Exacerbation With Fever, and Striatal Signal ChangesEmerging Phenotype of DYT-PRKRA
    Suvorit Subhas Bhowmick, Sarbani Raha, Amita Bohora
    • Full Text
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Resident & Fellow Section

  • Child Neurology: Initial Presentation of PCDH19-Related Epilepsy With New-Onset Refractory Status Epilepticus and Treatment With Anakinra
    Robin T. Varughese, Shefali Karkare, Annapurna Poduri, Sanjeev V. Kothare
    • Abstract
    • Full Text
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  • Pearls & Oy-sters: Vertical Diplopia and Ocular TorsionPeripheral vs Central Localization
    Manali Shah, Christopher T. Primiani, Amir Kheradmand, Kemar E. Green
    • Abstract
    • Full Text
    • Full Text (PDF)
  • Teaching NeuroImage: Cerebral Amyloid Angiopathy–Related InflammationAn Interesting Evolution of Imaging Findings
    Alex Dudley, Kieron Sweeney, Seamus Looby, Michael Farrell, Eavan McGovern
    • Full Text
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Disputes & Debates: Editors' Choice

  • Editors' Note: Early Neurologic Deterioration in Lacunar Stroke: Clinical and Imaging Predictors and Association With Long-term Outcome
    James E. Siegler, Steven Galetta
    • Full Text
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  • Reader Response: Early Neurologic Deterioration in Lacunar Stroke: Clinical and Imaging Predictors and Association With Long-term Outcome
    Jack Nathaniel Alpert
    • Full Text
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  • Reader Response: Early Neurologic Deterioration in Lacunar Stroke: Clinical and Imaging Predictors and Association With Long-term Outcome
    Andrea Zini, Umberto Pensato, Stefano Forlivesi
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