Postinfectious SARS-CoV-2 Opsoclonus-Myoclonus-Ataxia Syndrome

Update Item Information
Title Postinfectious SARS-CoV-2 Opsoclonus-Myoclonus-Ataxia Syndrome
Creator Jodi L. Nelson; Gregory M. Blume; Saurabh K. Bansal; Jacqueline R. Kaufman; Florence R. Woods; Xiaojun Zhang; Jorge C. Kattah
Affiliation Department of Neurology (JN, GB, FW, XZ, and JK), University of Illinois College of Medicine Peoria, Illinois Neurologic Institute, OSF St. Francis Medical Center, Peoria, Illinois; Department of Neurology (JK and FW), Illinois Neurologic Institute OSF St. Francis Medical Center, Peoria, Illinois; and Department of Internal Medicine (SB), University of Illinois College of Medicine Peoria, OSF St. Francis Medical Center, Peoria, Illinois
Abstract Background: The opsoclonus-myoclonus-ataxia syndrome (OMAS) represents a pathophysiology and diagnostic challenge. Although the diverse etiologies likely share a common mechanism to generate ocular, trunk, and limb movements, the underlying cause may be a paraneoplastic syndrome, as the first sign of cancer, or may be a postinfectious complication, and thus, the outcome depends on identifying the trigger mechanism. A recent hypothesis suggests increased GABAA receptor sensitivity in the olivary-oculomotor vermis-fastigial nucleus-premotor saccade burst neuron circuit in the brainstem. Therefore, OMAS management will focus on immunosuppression and modulation of GABAA hypersensitivity with benzodiazepines. Methods: We serially video recorded the eye movements at the bedside of 1 patient with SARS-CoV-2-specific Immunoglobulin G (IgG) serum antibodies, but twice-negative nasopharyngeal reverse transcription polymerase chain reaction (RT-PCR). We tested cerebrospinal fluid (CSF), serum, and nasopharyngeal samples. After brain MRI and chest, abdomen, and pelvis CT scans, we treated our patient with clonazepam and high-dose Solu-MEDROL, followed by a rituximab infusion after her formal eye movement analysis 10 days later. Results: The recordings throughout her acute illness demonstrated different eye movement abnormalities. While on high-dose steroids and clonazepam, she initially had macrosaccadic oscillations, followed by brief ocular flutter during convergence the next day; after 10 days, she had bursts of opsoclonus during scotopic conditions with fixation block but otherwise normal eye movements. Concern for a suboptimal response to high-dose Solu-MEDROL motivated an infusion of rituximab, which induced remission. An investigation for a paraneoplastic etiology was negative. CSF testing showed elevated neuron-specific enolase. Serum IgG to Serum SARS-CoV2 IgG was elevated with negative RT-PCR nasopharyngeal testing. Conclusion: A recent simulation model of macrosaccadic oscillations and OMAS proposes a combined pathology of brainstem and cerebellar because of increased GABAA receptor sensitivity. In this case report, we report 1 patient with elevated CSF neuronal specific enolase, macrosaccadic oscillations, ocular flutter, and OMAS as a SARS-CoV-2 postinfectious complication. Opsoclonus emerged predominantly with fixation block and suppressed with fixation, providing support to modern theories on the mechanism responsible for these ocular oscillations involving cerebellar-brainstem pathogenesis.
Subject COVID-19; Cerebellar Ataxia; Clonazepam; Immunoglobulin G; Methylprednisolone Hemisuccinate; Ocular Motility Disorders; Opsoclonus-Myoclonus Syndrome; Viral RNA;, GABA-A Receptors; Rituximab; SARS-CoV-2
OCR Text Show
Date 2022-06
Language eng
Format application/pdf
Type Text
Publication Type Journal Article
Source Journal of Neuro-Ophthalmology, June 2023, Volume 43, Issue 2
Collection Neuro-Ophthalmology Virtual Education Library: Journal of Neuro-Ophthalmology Archives: https://novel.utah.edu/jno/
Publisher Lippincott, Williams & Wilkins
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah
Rights Management © North American Neuro-Ophthalmology Society
ARK ark:/87278/s6v8d1f6
Setname ehsl_novel_jno
ID 2307904
Reference URL https://collections.lib.utah.edu/ark:/87278/s6v8d1f6
Back to Search Results