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Spotlight on the August 2 Issue.
Merino, Jose G. MD, MPhil; Editor-in-Chief, Neurology(R)
doi : 10.1212/WNL.0000000000200877
Volume 99(5) pgs. 177-219,e430-e548 August 2, 2022
What's in a Domain? The Role of NOTCH3 EGFr Domains in CADASIL Disease Severity.
Hack, Remco MD; Rutten, Julie MD, PhD; Lesnik Oberstein, Saskia A.J. MD, PhD
Advances and Remaining Challenges in Distinguishing Variants of Primary Progressive Aphasia.
Hillis, Argye Elizabeth MD, MA; Ruch, Kristina
Greater Efficacy of Avalglucosidase vs Alglucosidase Alfa in Adult Pompe Disease?: The Jury Is Still Out.
de Visser, Marianne PhD; Argov, Zohar PhD
Association of NOTCH3 Variant Position With Stroke Onset and Other Clinical Features Among Patients With CADASIL.
Cho, Bernard P.H. BSc, MSc; Jolly, Amy A. BSc, MSc; Nannoni, Stefania MD, PhD; Tozer, Daniel PhD; Bell, Steven PhD; Markus, Hugh S. DM, FMed Sci
Prevalence and Significance of the Vessel-Cluster Sign on Susceptibility-Weighted Imaging in Patients With Severe Small Vessel Disease.
Rudilosso, Salvatore MD, PhD; Chui, Ernest BmedSci; Stringer, Michael S. PhD; Thrippleton, Michael PhD; Chappell, Francesca PhD; Blair, Gordon W. MBChB; Garcia, Daniela Jaime MSc; Doubal, Fergus MBChB, PhD; Hamilton, Iona DCR; Kopczak, Anna MD; Ingrisch, Michael PhD; Kerkhofs, Danielle PhD; Backes, Walter H. PhD; Staals, Julie MD, PhD; van Oostenbrugge, Robert MD, PhD; Duering, Marco MD; Dichgans, Martin MD; Wardlaw, Joanna M. MD; on behalf of the SVDs@Target Investigators
Prevalence and Predictors of Vascular Cognitive Impairment in Patients With CADASIL.
Jolly, Amy A. BSc, MSc; Nannoni, Stefania MD, PhD; Edwards, Hayley BSc, MPhil; Morris, Robin G. PhD; Markus, Hugh S. DM, FMed Sci
Association of APOE-Independent Alzheimer Disease Polygenic Risk Score With Brain Amyloid Deposition in Asymptomatic Older Adults.
Xicota, Laura PhD; Gyorgy, Beata MSc; Grenier-Boley, Benjamin MSc; Lecoeur, Alexandre MSc; Fontaine, Gaelle MSc; Danjou, Fabrice PhD; Gonzalez, Jorge Samper PhD; Colliot, Olivier PhD; Amouyel, Philippe MD, PhD; Martin, Garance MSc; Levy, Marcel MD; Villain, Nicolas MD, PhD; Habert, Marie-Odile MD, PhD; Dubois, Bruno MD, PhD; Lambert, Jean-Charles PhD; Potier, Marie-Claude PharmD, PhD; on behalf of the INSIGHT pre-AD study group and for the Alzheimer's Disease Neuroimaging Initiative
[beta]-Amyloid-Dependent and -Independent Genetic Pathways Regulating CSF Tau Biomarkers in Alzheimer Disease.
Kumar, Atul PhD; Janelidze, Shorena PhD; Stomrud, Erik MD, PhD; Palmqvist, Sebastian MD, PhD; Hansson, Oskar MD, PhD *; Mattsson-Carlgren, Niklas MD, PhD *; for the Alzheimer's Disease Neuroimaging Initiative
Sensitivity of the Social Behavior Observer Checklist to Early Symptoms of Patients With Frontotemporal Dementia.
Toller, Gianina PhD; Cobigo, Yann PhD; Ljubenkov, Peter A. MD; Appleby, Brian S. MD; Dickerson, Bradford C. MD; Domoto-Reilly, Kimiko MD; Fong, Jamie C. MS; Forsberg, Leah K. PhD; Gavrilova, Ralitza H. MD; Ghoshal, Nupur PhD; Heuer, Hilary W. PhD; Knopman, David S. MD; Kornak, John PhD; Lapid, Maria I. MD; Litvan, Irene MD; Lucente, Diane E. MS; Mackenzie, Ian R. MD; McGinnis, Scott M. MD; Miller, Bruce L. MD; Pedraza, Otto PhD; Rojas, Julio C. MD, PhD; Staffaroni, Adam M. PhD; Wong, Bonnie PhD; Wszolek, Zbigniew K. MD; Boeve, Brad F. MD; Boxer, Adam L. MD, PhD; Rosen, Howard J. MD; Rankin, Katherine P. PhD; and the ALLFTD research consortium
Automated Detection of Speech Timing Alterations in Autopsy-Confirmed Nonfluent/Agrammatic Variant Primary Progressive Aphasia.
Garcia, Adolfo M. PhD *; Welch, Ariane E. MA *; Mandelli, Maria Luisa PhD; Henry, Maya L. PhD; Lukic, Sladjana PhD; Torres Prioris, Maria Jose PhD; Deleon, Jessica MD; Ratnasiri, Buddhika M. MA; Lorca-Puls, Diego L. PhD; Miller, Bruce L. MD; Seeley, William MD; Vogel, Adam P. PhD; Gorno-Tempini, Maria Luisa MD, PhD
Presymptomatic Lesion in Childhood Cerebral Adrenoleukodystrophy: Timing and Treatment.
Mallack, Eric James MD *; Van Haren, Keith P. MD *; Torrey, Anna BS; van de Stadt, Stephanie MD; Engelen, Marc MD, PhD; Raymond, Gerald V. MD; Fatemi, Ali MD; Eichler, Florian S. MD
Sensory Phenotypes for Balance Dysfunction After Mild Traumatic Brain Injury.
Fino, Peter C. PhD; Dibble, Leland E. PT, PhD, ATC; Wilde, Elisabeth A. PhD; Fino, Nora F. MS; Johnson, Paula PhD; Cortez, Melissa M. DO; Hansen, Colby R. MD; van der Veen, Susanne M. PhD; Skop, Karen M. PT, DPT, MS; Werner, J. Kent MD, PhD; Tate, David F. PhD; Levin, Harvey S. PhD; Pugh, Mary Jo V. PhD, RN; Walker, William C. MD
Long-term Safety and Efficacy of Avalglucosidase Alfa in Patients With Late-Onset Pompe Disease.
Dimachkie, Mazen M. MD; Barohn, Richard J. MD; Byrne, Barry MD, PhD; Goker-Alpan, Ozlem MD; Kishnani, Priya S. MD; Ladha, Shafeeq MD; Laforet, Pascal MD, PhD; Mengel, Karl Eugen MD; Pena, Loren D.M. MD, PhD; Sacconi, Sabrina MD, PhD; Straub, Volker MD, PhD; Trivedi, Jaya MD; Van Damme, Philip MD; van der Ploeg, Ans T. MD, PhD; Vissing, John MD; Young, Peter MD; Haack, Kristina An MD; Foster, Meredith ScD; Gilbert, Jane M. BSc; Miossec, Patrick MD; Vitse, Olivier PhD; Zhou, Tianyue PhD; Schoser, Benedikt MD; on behalf of the NEO-EXT investigators
[alpha]-Synuclein Seed Amplification Assays for Diagnosing Synucleinopathies: The Way Forward.
Bellomo, Giovanni PhD; De Luca, Chiara Maria Giulia MSc; Paoletti, Federico Paolini MD, PhD; Gaetani, Lorenzo MD, PhD; Moda, Fabio PhD; Parnetti, Lucilla MD, PhD
Early-Onset Dystonia, Exacerbation With Fever, and Striatal Signal Changes: Emerging Phenotype of DYT-PRKRA.
Bhowmick, Suvorit Subhas MD, DM; Raha, Sarbani MD; Bohora, Amita MD
Child Neurology: Initial Presentation of PCDH19-Related Epilepsy With New-Onset Refractory Status Epilepticus and Treatment With Anakinra.
Varughese, Robin T. MD; Karkare, Shefali MD; Poduri, Annapurna MD; Kothare, Sanjeev V. MD
Pearls & Oy-sters: Vertical Diplopia and Ocular Torsion: Peripheral vs Central Localization.
Shah, Manali MD; Primiani, Christopher T. MD; Kheradmand, Amir MD; Green, Kemar E. DO
Teaching NeuroImage: Cerebral Amyloid Angiopathy-Related Inflammation: An Interesting Evolution of Imaging Findings.
Dudley, Alex MB BCh, BAO; Sweeney, Kieron MB BCh, BAO, MD, FRCSI (SN); Looby, Seamus MB BCh, BAO, FRCPI, FFR RCSI; Farrell, Michael FRCPI, FRCPath, FRCPC; McGovern, Eavan MB BCh, BAO, PhD
Editors' Note: Early Neurologic Deterioration in Lacunar Stroke: Clinical and Imaging Predictors and Association With Long-term Outcome.
Siegler, James E. MD; Galetta, Steven MD
Reader Response: Early Neurologic Deterioration in Lacunar Stroke: Clinical and Imaging Predictors and Association With Long-term Outcome.
Alpert, Jack Nathaniel
Reader Response: Early Neurologic Deterioration in Lacunar Stroke: Clinical and Imaging Predictors and Association With Long-term Outcome.
Zini, Andrea; Pensato, Umberto; Forlivesi, Stefano
