Annals of Neurology




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Annals of Neurology: Volume 93, Number 6, June 2023

doi : 10.1002/ana.26395

Volume 93, Issue 6

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Time for the Next Step in Clinical Trial Evolution?

Erin E. Longbrake MD, PhD

doi : 10.1002/ana.26655

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Ravulizumab in Aquaporin-4–Positive Neuromyelitis Optica Spectrum Disorder

Sean J. Pittock MD, Michael Barnett MD, PhD, Jeffrey L. Bennett MD, PhD, Achim Berthele MD, Jérôme de Sèze PhD, Michael Levy MD, PhD, Ichiro Nakashima MD, PhD, Celia Oreja-Guevara MD, PhD, Jacqueline Palace DM, Friedemann Paul MD, Carlo Pozzilli MD, PhD, Marcus Yountz MD, Kerstin Allen MA, Yasmin Mashhoon PhD, Ho Jin Kim MD, PhD

doi : 10.1002/ana.26626

CHAMPION-NMOSD (NCT04201262) is a phase 3, open-label, externally controlled interventional study evaluating the efficacy and safety of the terminal complement inhibitor ravulizumab in adult patients with anti–aquaporin-4 antibody–positive (AQP4+) neuromyelitis optica spectrum disorder (NMOSD).

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Aquaporin-4 Antibody Dynamics and Relapse Risk in Seropositive Neuromyelitis Optica Spectrum Disorder Treated with Immunosuppressants

He-Xiang Yin MD, Ying-Jie Wang MD, Man-Ge Liu MD, Ding-Ding Zhang PhD, Hai-Tao Ren BS, Zhi-Feng Mao PhD, Yao Zhang MD, Bin Peng MD, Li-Ying Cui MD, Yan Xu MD, PhD

doi : 10.1002/ana.26623

To investigate aquaporin-4 antibody (AQP4-IgG) dynamics and relapse risk in patients with seropositive neuromyelitis optica spectrum disorder treated with immunosuppressants.

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Ultra-Low Level Somatic Mutations and Structural Variations in Focal Cortical Dysplasia Type II

Ja Hye Kim MD, PhD, Ji-Hyung Park MD, Junehawk Lee PhD, Jung Woo Park PhD, Hyun Jung Kim MD, PhD, Won Seok Chang MD, PhD, Dong-Seok Kim MD, PhD, Young Seok Ju MD, PhD, Eleonora Aronica PhD, Jeong Ho Lee MD, PhD

doi : 10.1002/ana.26609

Brain somatic mutations in mTOR pathway genes are a major genetic etiology of focal cortical dysplasia type II (FCDII). Despite a greater ability to detect low-level somatic mutations in the brain by deep sequencing and analytics, about 40% of cases remain genetically unexplained.

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Stroke in Patients with Bacterial Meningitis: A Cohort Study and Meta-Analysis

Carolin Beuker MD, Nils Werring MD, Nadine Bonberg, Jan-Kolja Strecker PhD, Antje Schmidt-Pogoda MD, Wolfram Schwindt MD, Paul Stracke MD, Andreas Schulte-Mecklenbeck PhD, Catharina Gross PhD, Heinz Wiendl MD, Heike Minnerup MD, Jens Minnerup MD

doi : 10.1002/ana.26618

The purpose of this study was to characterize patients with ischemic stroke due to bacterial meningitis.

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Effect of Alteplase on Ischemic Stroke Mortality Is Dependent on Stroke Severity

Adam de Havenon MD, MS, Mehdi Abbasi MD, Shadi Yaghi MD, Alen Delic MS, Aaron Bangad BS, Karen Johnston MD, MSc, David Tirschwell MD, MS, Kevin N. Sheth MD

doi : 10.1002/ana.26621

Although intravenous alteplase (IV-tPA) has a beneficial effect on functional outcome after ischemic stroke (IS), prior studies of IV-tPA's impact on post-stroke mortality did not have sufficient representation of more severe stroke.

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Determinants of Infarct Core Growth During Inter-hospital Transfer for Thrombectomy

Pierre Seners MD, PhD, Lauranne Scheldeman MD, Soren Christensen PhD, Michael Mlynash MD, Adrien Ter Schiphorst MD, Caroline Arquizan MD, Vincent Costalat MD, Hilde Henon MD, Martin Bretzner MD, Jeremy J. Heit MD, PhD, Jean-Marc Olivot MD, PhD, Maarten G. Lansberg MD, PhD, Gregory W. Albers MD, on behalf of the Infarct-Growth Collaborators

doi : 10.1002/ana.26613

Patients with acute ischemic stroke harboring a large vessel occlusion who present to primary stroke centers often require inter-hospital transfer for thrombectomy. We aimed to determine clinical and imaging factors independently associated with fast infarct growth (IG) during inter-hospital transfer.

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Cerebral Small Vessel Disease Progression Increases Risk of Incident Parkinsonism

Mina A. Jacob MD, Mengfei Cai MD, PhD, Mayra Bergkamp MD, Sirwan K. L. Darweesh MD, PhD, Liza M. Y. Gelissen MD, José Marques PhD, David G. Norris PhD, Marco Duering MD, Rianne A. J. Esselink MD, PhD, Anil M. Tuladhar MD, PhD, Frank-Erik de Leeuw MD, PhD

doi : 10.1002/ana.26615

Cerebral small vessel disease (SVD) is associated with motor impairments and parkinsonian signs cross-sectionally, however, there are little longitudinal data on whether SVD increases risk of incident parkinsonism itself. We investigated the relation between baseline SVD severity as well as SVD progression, and incident parkinsonism over a follow-up of 14 years.

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Stroke and Acute Coronary Syndrome from One Clot

Marialuisa Zedde MD, Rosario Pascarella MD

doi : 10.1002/ana.26646

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Circadian Rest–Activity Rhythms, Delirium Risk, and Progression to Dementia

Lei Gao MBBS, Peng Li PhD, Nicole Gaykova BS, Xi Zheng MS, Chenlu Gao PhD, Jacqueline M. Lane PhD, Richa Saxena PhD, Frank A. J. L. Scheer PhD, Martin K. Rutter MD, Oluwaseun Akeju MD, Kun Hu PhD

doi : 10.1002/ana.26617

Delirium is a complex neurocognitive syndrome suspected to be bidirectionally linked to dementia. Circadian rhythm disturbances likely contribute to dementia pathogenesis, but whether these disturbances are related to delirium risk and progression to all-cause dementia is unknown.

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Change in Cerebrospinal Fluid Tau Microtubule Binding Region Detects Symptom Onset, Cognitive Decline, Tangles, and Atrophy in Dominantly Inherited Alzheimer's Disease

Kanta Horie PhD, Yan Li PhD, Nicolas R. Barthélemy PhD, Brian Gordon PhD, Jason Hassenstab PhD, Tammie L.S. Benzinger MD, PhD, Anne M. Fagan PhD, John C. Morris MD, Celeste M. Karch PhD, Chengjie Xiong PhD, Ricardo Allegri MD, Patricio Chrem Mendez MD, Takeshi Ikeuchi MD, PhD, Kensaku Kasuga MD, PhD, James Noble MD, Martin Farlow MD, Jasmeer Chhatwal MD, Gregory Day MD, Peter R. Schofield MD, Colin L. Masters MD, Johannes Levin MD, Mathias Jucker PhD, Jae-Hong Lee MD, PhD, Jee Hoon Roh MD, PhD, Chihiro Sato PhD, Pallavi Sachdev PhD, Akihiko Koyama PhD, Larisa Reyderman PhD, Randall J. Bateman MD, Eric McDade DO, and the Dominantly Inherited Alzheimer Network

doi : 10.1002/ana.26620

Identifying cerebrospinal fluid measures of the microtubule binding region of tau (MTBR-tau) species that reflect tau aggregation could provide fluid biomarkers that track Alzheimer's disease related neurofibrillary tau pathological changes. We examined the cerebrospinal fluid (CSF) MTBR-tau species in dominantly inherited Alzheimer's disease (DIAD) mutation carriers to assess the association with Alzheimer's disease (AD) biomarkers and clinical symptoms.

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Decreased Cerebrospinal Fluid Amyloid β 38, 40, 42, and 43 Levels in Sporadic and Hereditary Cerebral Amyloid Angiopathy

Anna M. De Kort MD, H. Bea Kuiperij PhD, Tainá M. Marques PhD, Lieke Jäkel PhD, Emma van den Berg MSc, Iris Kersten BSc, Hugo E. P. van Berckel-Smit MD, Marco Duering MD, PhD, Erik Stoops MSc, Wilson F. Abdo MD, PhD, Ingeborg Rasing MD, Sabine Voigt MSc, Emma A. Koemans MD, Kanishk Kaushik, Andrew Davock Warren BSc, Steven M. Greenberg MD, PhD, Gunnar Brinkmalm PhD, Gisela M. Terwindt MD, PhD, Marieke J. H. Wermer MD, PhD, Floris H. B. M. Schreuder MD, PhD, Catharina J. M. Klijn MD, PhD, Marcel M. Verbeek PhD

doi : 10.1002/ana.26610

Vascular amyloid β (Aβ) accumulation is the hallmark of cerebral amyloid angiopathy (CAA). The composition of cerebrospinal fluid (CSF) of CAA patients may serve as a diagnostic biomarker of CAA. We studied the diagnostic potential of the peptides Aβ38, Aβ40, Aβ42, and Aβ43 in patients with sporadic CAA (sCAA), hereditary Dutch-type CAA (D-CAA), and Alzheimer disease (AD).

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Biological Age Acceleration and Motoric Cognitive Risk Syndrome

Sanish Sathyan PhD, Emmeline Ayers MPH, Dristi Adhikari MA, Tina Gao MPH, Sofiya Milman MD, Nir Barzilai MD, Joe Verghese MBBS, MS

doi : 10.1002/ana.26624

Motoric cognitive risk (MCR) syndrome, a predementia syndrome characterized by slow gait and subjective cognitive concerns, is associated with multiple age-related risk factors. We hypothesized that MCR is associated with biological age acceleration. We examined the associations of biological age acceleration with MCR, and mortality risk in MCR cases.

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Multisite Hebbian Plasticity Restores Function in Humans with Spinal Cord Injury

Hang Jin Jo PhD, Ethan Kizziar BS, Sina Sangari PhD, David Chen MD, Allison Kessler MD, Ki Kim MD, Alan Anschel MD, Allen W. Heinemann PhD, Brett D. Mensh PhD, Saria Awadalla PhD, Richard L. Lieber PhD, Martin Oudega PhD, Monica A. Perez PhD

doi : 10.1002/ana.26622

Spinal cord injury (SCI) damages synaptic connections between corticospinal axons and motoneurons of many muscles, resulting in devastating paralysis. We hypothesized that strengthening corticospinal-motoneuronal synapses at multiple spinal cord levels through Hebbian plasticity (i.e., “neurons that fire together, wire together�) promotes recovery of leg and arm function.

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Adolescent Headache Due to Congenital Pelvic/Sacral Vascular Malformation

Wouter I Schievink MD, Marcel M Maya MD, Alexandra J Borst MD

doi : 10.1002/ana.26651

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MOG-IgG-Associated Bilateral Optic Neuritis in Temporal Relation to Monkeypox Vaccination

Daniel Engels MD, PhD, Simone Mader PhD, Stefanie Förderreuther MD, Markus Reindl MD, Joachim Havla MD, Edgar Meinl MD, Tania Kümpfel MD, Lisa Ann Gerdes MD

doi : 10.1002/ana.26664

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Reply to “MOG-IgG-Associated Bilateral Optic Neuritis in Temporal Relation to Monkeypox Vaccination�

Kelli M. Money MD, PhD, Takashi Kitani MD, Daniel M. Pastula MD, MHS

doi : 10.1002/ana.26660

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Is there an Association between Tuber Involvement of the Fusiform Face Area in Autism Diagnosis?

Kevin S. Weiner PhD, Ethan H. Willbrand BA

doi : 10.1002/ana.26632

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Reply to “Is There an Association between Tuber Involvement of the Fusiform Face Area in Autism Diagnosis?�

Alexander L. Cohen MD, PhD, Mallory R. Kroeck MA, Michael D. Fox MD, PhD, on behalf of the TACERN Study Group

doi : 10.1002/ana.26634

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