March 2020 Resident & Fellow Rounds NPub.org/rf

John J. Millichap, MD, Editor, Roy E. Strowd III, MD, Deputy Editor

A summary of recently published articles in the Neurology® Resident & Fellow Section

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March 3, 2020, issue MORE ONLINE This issue contains a Clinical Reasoning case featur- ing a 74-year-old man with bilateral foot drop. A Editor’s Blog Pearls & Oy-sters article showcases the rare pri- NPub.org/rfblog mary angiitis of the CNS. In a Teaching Neuro- Image, a young boy presents with a rare childhood-onset disease. Neurology Podcast NPub.org/rf Clinical Reasoning: A case of bilateral foot drop in a 74-year-old man fi This case reviews the key clinical ndings to be considered when examining a patient with Resident & Fellow bilateral foot drop. Clinical Reasoning Page 405 Book NPub.org/crb Pearls & Oy-sters: Primary angiitis of the CNS presenting with Resident & Fellow recurrent intracranial hemorrhage e-Pearls A patient with acute onset of left hemiparesis, numbness, and dysarthria is presented. The case NPub.org/epearls showcases the diagnostic challenges of rare disease presentation. Page e992 Resident & Fellow Child ff Neurology Book Teaching NeuroImages: Hopkins syndrome: A rare di erential NPub.org/cnbook diagnosis of neurogenic monomelic Hopkins syndrome is a rare disorder presenting with acute flaccid paralysis and can mimic monomelic amyotrophy. This Teaching NeuroImage demonstrates findings in an 11-year-old boy who presented with left thigh weakness. Page e996

March 10, 2020, issue This issue contains two Clinical Reasoning articles. The first case is a teenage boy presenting with an immunologic disease that can cause progressive cognitive decline and gait impairment. The second is presented as one of our popular Mystery Cases and involves a middle-aged man with worsening , nausea, and vomiting. Finally, the differential diagnosis of pediatric arterial ischemic in a young girl with cardiac myxomas is explained in the Child Neurology case. Clinical Reasoning: A 16-year-old boy with progressive cognitive decline and gait impairment This case discusses the need for early diagnosis of hyperimmunoglobulin E syndrome for effective treatment. Page 449

588 Copyright © 2020 American Academy of Neurology Copyright © 2020 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. Child Neurology: Arterial ischemic stroke in Teaching Video NeuroImages: a 12-year-old patient with cardiac myxomas Intralabyrinthine schwannoma A 12-year-old girl had cardiac myxoma and developed pediatric masquerading as M´eni`eredisease arterial ischemic stroke. The patient showed aphasia, disori- This case discusses high-resolution MRI as part of the di- entation, and agitation. agnosis of intralabyrinthine schwannoma. Page e1103 Page e1227 Clinical Reasoning: A 63-year-old man with gastroenteritis progressing to stupor March 24, 2020, issue and quadriparesis This issue features 2 Clinical Reasoning cases. The first case fea- A patient with , nausea, and vomiting is presented. tures a man with super-refractory and the second The differential diagnosis and important considerations in the showcases a woman intubated at the hospital following a week of diagnosis of CLIPPERS (chronic lymphocytic inflammation fatigue and drowsiness. The Child Neurology case features a case with pontine perivascular enhancement responsive to steroids) of ethylmalonic with a novel variation in ETHE1. are discussed. Page e1107 Clinical Reasoning: Ketogenic diet in adult super-refractory status epilepticus March 17, 2020, issue This article details the case of a man developing super- refractory status epilepticus following a car accident. An al- This issue features an Education Research article on the present ternative treatment of ketogenic diet for super-refractory status state of fellowship training for neuroimmunology and multiple epilepticus is highlighted. sclerosis in the United States. The first Clinical Reasoning case Page 541 features a man with insomnia and psychiatric and motor symp- toms and the second case a woman with worsening neurologic Clinical Reasoning: A young healthy woman deficits, in whom neuropathologic features and genetic testing with difficult-to-wean acute ventilator were instrumental in making a diagnosis. The Teaching Video dependence NeuroImage case exhibits intralabyrinthine schwannoma. The patient with fatigue and drowsiness was intubated after hy- Education Research: and percapnic respiratory failure. MRI revealed Chiari malformation, which is a consideration for investigation of respiratory failure. neuroimmunology fellowship training status Page e1340 in the United States This article highlights the need for postgraduate training Child Neurology: Ethylmalonic in multiple sclerosis and neuroimmunology. The study encephalopathy showcases the need for widespread training across neu- A boy presented with developmental decline due to ethyl- roimmunologic diagnoses and cross-disciplinary training. malonic encephalopathy, a rare autosomal recessive disorder. Page 495 Exome sequencing revealed a variation of the ETHE1 gene. Page e1336 Clinical Reasoning: A 45-year-old man with progressive insomnia and psychiatric and motor symptoms March 31, 2020, issue Familial fatal insomnia, a rare autosomal dominant prion dis- This issue features a Mystery Case describing an 82-year-old ease, presents typically with attention issues, insomnia, and man presenting with tongue . Next, there is a Pearls and progressive . A patient presented with 6 months of Oy-sters article that describes the diagnosis of a middle-aged worsening neuropsychiatric and motor symptoms along with man with worsening gait and slurred speech. Finally, there are severe insomnia. two Teaching NeuroImages cases, one on trochlear nerve Page e1213 schwannoma and the other showing superior compression of Clinical Reasoning: A 42-year-old woman the left oculomotor nerve in a middle-aged woman. with progressive cognitive difficulties and Mystery Case: Symptomatic isolated tongue gait imbalance tremor of cortical origin due to stroke A 42-year-old woman with a history of psoriatic arthritis pre- An 82-year-old man presented with tongue tremor. This case ffi sented with di culty in concentration and task completion as indicates that spontaneous tongue tremor may occur following well as gait imbalance. cortical ischemic stroke. Page e1219 Page 591

Neurology.org/N Neurology | Volume 94, Number 13 | March 31, 2020 589 Copyright © 2020 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. Pearls & Oy-sters: Progressive ataxia and Teaching NeuroImages: Pupil-sparing palatal tremor: Imaging and disease course compression of oculomotor nerve by This case describes a man who came to clinic with 3 years of posterior cerebral artery vessel worsening gait and slurred speech. The case is unique in that Neuro-ophthalmic examination was performed on a woman the patient experienced spontaneous resolution of tremor but presenting a 30-year history of worsening left blepharoptosis. worsening gait ataxia. Page e1450 Page e1445 Teaching NeuroImages: Trochlear nerve schwannoma The case of a woman with binocular vertical diplopia on downgaze is presented. Page e1448

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590 Neurology | Volume 94, Number 13 | March 31, 2020 Neurology.org/N Copyright © 2020 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. Resident & Fellow Rounds John J. Millichap and Roy E. Strowd III Neurology 2020;94;588-590 DOI 10.1212/WNL.0000000000009176

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Neurology ® is the official journal of the American Academy of Neurology. Published continuously since 1951, it is now a weekly with 48 issues per year. Copyright © 2020 American Academy of Neurology. All rights reserved. Print ISSN: 0028-3878. Online ISSN: 1526-632X.