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

Jodi L Nelson1, Gregory M Blume, Saurabh K Bansal

  • 1Department 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.

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Opsoclonus-myoclonus-ataxia syndrome (OMAS) can be a post-SARS-CoV-2 complication. This case highlights OMAS with elevated neuron-specific enolase, successfully treated with rituximab.

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Area of Science:

  • Neuroscience
  • Immunology
  • Infectious Disease

Background:

  • Opsoclonus-myoclonus-ataxia syndrome (OMAS) presents diagnostic challenges due to diverse etiologies, potentially linked to paraneoplastic syndromes or postinfectious complications.
  • A hypothesis suggests increased GABAA receptor sensitivity in the olivary-oculomotor vermis-fastigial nucleus-premotor saccade burst neuron circuit.
  • Management strategies include immunosuppression and GABAA modulation with benzodiazepines.

Observation:

  • A patient with SARS-CoV-2 antibodies and negative PCR developed OMAS, characterized by macrosaccadic oscillations, ocular flutter, and opsoclonus.
  • Cerebrospinal fluid (CSF) revealed elevated neuron-specific enolase.
  • Eye movement abnormalities evolved during treatment with steroids and clonazepam.

Findings:

  • The patient exhibited evolving eye movement abnormalities, including opsoclonus predominantly with fixation block.
  • Treatment with high-dose Solu-MEDROL and clonazepam was followed by rituximab infusion, inducing remission.
  • Elevated serum IgG to SARS-CoV-2 and CSF neuron-specific enolase supported a post-SARS-CoV-2 etiology.

Implications:

  • This case supports the hypothesis of a combined brainstem and cerebellar pathology due to increased GABAA receptor sensitivity in OMAS.
  • It underscores the potential of SARS-CoV-2 as a trigger for OMAS, necessitating investigation of post-infectious complications.
  • Effective management involved immunosuppression with rituximab, highlighting its role in refractory cases.