Neurology

Fifer, Matthew S. PhD*; McMullen, David P. MD*; Osborn, Luke E. PhD; Thomas, Tessy M. PhD; Christie, Breanne PhD; Nickl, Robert W. PhD; Candrea, Daniel N. BS; Pohlmeyer, Eric A. PhD; Thompson, Margaret C. PhD; Anaya, Manuel A. MD; Schellekens, Wouter PhD; Ramsey, Nick F. PhD; Bensmaia, Sliman J. PhD; Anderson, William S. MD, PhD; Wester, Brock A. PhD; Crone, Nathan E. MD; Celnik, Pablo A. MD; Cantarero, Gabriela L. PhD; Tenore, Francesco V. PhD

doi : 10.1212/WNL.0000000000013173

Volume 98(7), 15 February 2022, p e679-e687

The restoration of touch to fingers and fingertips is critical to achieving dexterous neuroprosthetic control for individuals with sensorimotor dysfunction. However, localized fingertip sensations have not been evoked via intracortical microstimulation (ICMS).

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Validation of Plasma Amyloid-[beta] 42/40 for Detecting Alzheimer Disease Amyloid Plaques

Li, Yan PhD; Schindler, Suzanne E. PhD; Bollinger, James G. PhD; Ovod, Vitaliy MS; Mawuenyega, Kwasi G. PhD; Weiner, Michael W. MD; Shaw, Leslie M. PhD; Masters, Colin L. MD; Fowler, Christopher J. PhD; Trojanowski, John Q. PhD; Korecka, Magdalena PhD; Martins, Ralph N. PhD; Janelidze, Shorena PhD; Hansson, Oskar MD, PhD; Bateman, Randall J. MD

doi : 10.1212/WNL.0000000000013211

Volume 98(7), 15 February 2022, p e688-e699

To determine the diagnostic accuracy of a plasma A[beta]42/A[beta]40 assay in classifying amyloid PET status across global research studies using samples collected by multiple centers that utilize different blood collection and processing protocols.

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Association of Cognitive Impairment With Free Water in the Nucleus Basalis of Meynert and Locus Coeruleus to Transentorhinal Cortex Tract

Chu, Winston Thomas PhD; Wang, Wei-en PhD; Zaborszky, Laszlo MD, PhD; Golde, Todd Eliot MD, PhD; DeKosky, Steven MD; Duara, Ranjan MD; Loewenstein, David A. PhD; Adjouadi, Malek PhD; Coombes, Stephen A. PhD; Vaillancourt, David E. PhD

doi : 10.1212/WNL.0000000000013206

Volume 98(7), 15 February 2022, p e700-e710

The goal of this work was to determine the relationship between diffusion microstructure and early changes in Alzheimer disease (AD) severity as assessed by clinical diagnosis, cognitive performance, dementia severity, and plasma concentrations of neurofilament light chain.

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Associations of Chronic Kidney Disease With Dementia Before and After TIA and Stroke: Population-Based Cohort Study

Kelly, Dearbhla M. MBBCh, DPhil, MRCPI; Pendlebury, Sarah T. FRCP, DPhil; Rothwell, Peter M. MD, PhD, FRCP, FMedSci

doi : 10.1212/WNL.0000000000013205

Volume 98(7), 15 February 2022, p e711-e720

Individuals with chronic kidney disease (CKD) appear to be at increased risk of cognitive impairment, with both vascular and neurodegenerative mechanisms postulated. To explore the vascular hypothesis, we studied the association between CKD and dementia before and after TIA and stroke.

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Effect of Age at Pediatric Stroke on Long-term Cognitive Outcome

Abgottspon, Stephanie MSc; Thaqi, Qendresa PhD; Steiner, Leonie PhD; Slavova, Nedelina MD; Grunt, Sebastian MD, PhD; Steinlin, Maja MD; Everts, Regula PhD

doi : 10.1212/WNL.0000000000013207

Volume 98(7), 15 February 2022, p e721-e729

To investigate the effect of age at pediatric arterial ischemic stroke on long-term cognitive outcome in order to identify patients particularly at risk for the development of long-term cognitive sequelae.

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Phenotypic Spectrum of Dystrophinopathy Due to Duchenne Muscular Dystrophy Exon 2 Duplications

Zambon, Alberto A. MD, PhD*; Waldrop, Megan A. MD*; Alles, Roxane PhD; Weiss, Robert B. PhD; Conroy, Sara BS; Moore-Clingenpeel, Melissa MS; Previtali, Stefano MD, PhD+; Flanigan, Kevin M. MD+; on behalf of the Italian DMD Network and the United Dystrophinopathy Project

doi : 10.1212/WNL.0000000000013246

Volume 98(7), 15 February 2022, p e730-e738

To describe the phenotypic spectrum of dystrophinopathy in a large cohort of individuals with DMD exon 2 duplications (Dup2), who may be particularly amenable to therapies directed at restoring expression of either full-length dystrophin or nearly full-length dystrophin through utilization of the DMD exon 5 internal ribosome entry site (IRES).

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Dermatomyositis: Muscle Pathology According to Antibody Subtypes

Tanboon, Jantima MD; Inoue, Michio MD, PhD; Saito, Yoshihiko MD, PhD; Tachimori, Hisateru PhD; Hayashi, Shinichiro PhD; Noguchi, Satoru PhD; Okiyama, Naoko MD, PhD; Fujimoto, Manabu MD, PhD; Nishino, Ichizo MD, PhD

doi : 10.1212/WNL.0000000000013176

Volume 98(7), 15 February 2022, p e739-e749

Discoveries of dermatomyositis-specific antibodies (DMSAs) in patients with dermatomyositis raised awareness of various myopathologic features among antibody subtypes. However, only perifascicular atrophy and perifascicular myxovirus resistant protein A (MxA) overexpression were officially included as definitive pathologic criteria for dermatomyositis classification. We aimed to demonstrate myopathologic features in MxA-positive dermatomyositis to determine characteristic myopathologic features in different DMSA subtypes.

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In Vivo Reflectance Microscopy of Meissner Corpuscles and Bedside Measures of Large Fiber Sensory Function: A Normative Data Cohort

Creigh, Peter D. MD*; Du, Khai BS*; Wood, Elizabeth P. MA; Mountain, Joan BS; Sowden, Janet BSc; Charles, Julie AAS; Behrens-Spraggins, Steffen BS; Herrmann, David N. MBBCh

doi : 10.1212/WNL.0000000000013175

Volume 98(7), 15 February 2022, p e750-e758

The goal of this work was to establish age-, sex-, and body dimension-adjusted normal cutoff values for Meissner corpuscle (MC) densities via in vivo reflectance confocal microscopy (RCM), timed vibration sensory thresholds with a 128-Hz tuning fork, and touch-pressure sensory thresholds with standardized monofilaments for clinical and research application.

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Age-Stratified Risk of Cerebral Venous Sinus Thrombosis After SARS-CoV-2 Vaccination

Krzywicka, Katarzyna MD, MPhil*; van de Munckhof, Anita MD*; Sanchez van Kammen, Mayte MD; Heldner, Mirjam R. MD, MSc; Jood, Katarina MD, PhD; Lindgren, Erik MD; Tatlisumak, Turgut MD, PhD; Putaala, Jukka MD, PhD; Kremer Hovinga, Johanna A. MD; Middeldorp, Saskia MD, PhD; Levi, Marcel M. MD, PhD; Cordonnier, Charlotte MD, PhD; Arnold, Marcel MD; Zwinderman, Aeilko H. MD, PhD; Ferro, Jose M. MD, PhD; Coutinho, Jonathan M. MD, PhD+; Aguiar de Sousa, Diana MD, PhD+

doi : 10.1212/WNL.0000000000013148

Volume 98(7), 15 February 2022, p e759-e768

Cerebral venous sinus thrombosis (CVST) as a part of the thrombosis and thrombocytopenia syndrome is a rare adverse drug reaction of severe acute respiratory syndrome coronavirus disease 2 (SARS-CoV-2) vaccination. Estimated background rate of CVST with thrombocytopenia is 0.1 per million per month. We assessed the age-stratified risk of CVST with and without thrombocytopenia after SARS-CoV-2 vaccination.

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What Are the Interactions Between the Midbrain Dopamine System in Pain?

Benarroch, Eduardo MD

doi : 10.1212/WNL.0000000000013253

Volume 98(7), 15 February 2022, p 274-278

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Accelerated Implementation of a Virtual Neurology Clerkship Amid a Global Crisis

Govindarajan, Raghav MD; Vu, Anh-Thu N. MD, MS; Salas, Rachel Marie E. MD, MEd; Miller, Alexandra Michelle MD, PhD; Sandness, David J. MD; Said, Rana R. MD; Southerland, Andrew M. MD, MSc; Fernandez, Andres MD, MSEd; Romano, Sofia BS; Sennott, Brianna J. BS; Patino-Murillas, Jorge MD; Soni, Madhu MD; on behalf of the AAN Undergraduate Education Subcommittee and Education Committee

doi : 10.1212/WNL.0000000000013222

Volume 98(7), 15 February 2022, p 279-286

The standard neurology clinical experience in medical school focuses primarily on bedside patient encounters; however, the limitations of the clinical environment due to the current COVID-19 pandemic have accelerated the need for virtual curriculum development. To provide guidance to Neurology clerkship directors during this unprecedented time, the American Academy of Neurology (AAN) Undergraduate Education Subcommittee (UES) formed a workgroup to develop an outline for a virtual curriculum, provide recommendations, and describe models of integrating virtual curricula into the neurology clerkship. In this overview, we discuss different methods of virtual instruction, hybrid models of clerkship training and the challenges to its implementation, professionalism issues, and modification of feedback and assessment techniques specific to the virtual learning environment. We also offer suggestions for implementation of a hybrid virtual curriculum into the neurology clerkship. The virtual curriculum is intended to supplement the core neurology in-person clinical experience and should not be used for shortening or replacing the required neurology clinical clerkship.

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Seizing an Opportunity for Improvement

Guterman, Elan L. MD, MAS; Lowenstein, Daniel H. MD; Sporer, Karl A. MD

doi : 10.1212/WNL.0000000000013248

Volume 98(7), 15 February 2022, p 287-288

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SGCE Myoclonus-Dystonia: An Inherited Movement Disorder

Yoganathan, Sangeetha MD, DNB, DM; Kumar, Madhan MD, DCH; Sharma, Suvasini MD, DM; Patel, Smruti MD; Danda, Sumita MD, DM; Thomas, Maya MD, DM

doi : 10.1212/WNL.0000000000013209

Volume 98(7), 15 February 2022, p 289

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Sinus Pericranii With Dural Venous Lakes in a Woman With a Pulsatile Frontal Mass

Ojha, Piyush MBBS, MD, DM; Mahajan, Anshu MBBS, MD, DM; Pal, Tanmoy MBBS, MD, DNB; Dubey, Sudhir MBBS, MS, MCh; Goel, Gaurav MBBS, MD, DM

doi : 10.1212/WNL.0000000000013223

Volume 98(7), 15 February 2022, p 290-291

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Pearls & Oy-sters: Cerebral Abscess Secondary to Pulmonary Arteriovenous Malformation in Hereditary Hemorrhagic Telangiectasia

Roberts, Jodie I. MD, MSc; Woodward, Kristine MD, MSc; Kirton, Adam MD, MSc; Esser, Michael J. MD, PhD

doi : 10.1212/WNL.0000000000013181

Volume 98(7), 15 February 2022, p 292-295

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant condition that is linked to a myriad of neurologic complications arising from vascular malformations of the brain, spinal cord, and lungs. Our case describes a previously healthy 3-year-old male who presented to hospital with fever of unknown origin and was found to have a brain abscess stemming from a pulmonary arteriovenous malformation (PAVM). This etiology was identified after a period of diagnostic delay; the medical team was suspicious for a proximal embolic source due to the presence of multiple tiny infarcts seen on MRI of the brain, but transthoracic echocardiogram and head and neck angiogram were unremarkable. Fortunately, an enhanced CT of the chest was performed, identifying a moderately sized PAVM. PAVMs are associated with intracranial abscesses due to shunting and loss of the normal filtering effects of the lung capillary bed. Impaired pulmonary filtration can permit paradoxical thromboemboli and septic microemboli to enter systemic circulation, predisposing patients with PAVMs to cerebral abscess and ischemic stroke. Screening for PAVMs with contrast-enhanced echocardiogram or enhanced CT of the chest may be considered in patients with cryptogenic brain abscess or recurrent embolic stroke of unknown origin. PAVMs are often associated with hereditary hemorrhagic telangiectasia (HHT). As many features of HHT have delayed clinical manifestation, genetic testing for HHT should be considered in all people with PAVM, even in the absence of other clinical features. In our case, genetic testing returned positive, confirming a new diagnosis of HHT type 1.

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