Perplexing Initial Presentations of MOGAD in Two Children: Intracranial Hypertension and New-Onset Seizure

Daniel J. Zhou, Andria M. Powers, Caleb A. Cave, Emily K. Dickas, Mary C. Rickard, Geetanjali Rathore, Rhonda R. Wright, Rana K. Zabad, Sookyong Koh

Research output: Contribution to journalArticlepeer-review

Abstract

We report two distinct challenging initial presentations of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Case 1 describes a 12-year-old boy who developed headaches refractory to pain medication followed by cranial neuropathies and intracranial hypertension, confirmed by lumbar puncture with an opening pressure >36 cm H2O. Case 2 describes a 3-year-old boy who developed new-onset seizures refractory to antiseizure medications, a presentation of FLAIR-hyperintense lesions in MOG-antibody associated encephalitis with seizures (FLAMES). On repeat magnetic resonance imaging, both patients were found to have cortical T2 hyperintensities, leptomeningeal contrast enhancement, and bilateral optic nerve enhancement. In the cerebrospinal fluid, both patients had CSF pleocytosis with neutrophilic predominance. The patients were treated with intravenous immunoglobulins, plasma exchange, and high-dose corticosteroids. The first patient achieved disease remission, whereas the second patient required the addition of rituximab for management of seizures. The two cases highlight the pleomorphic clinical phenotypes of MOGAD.

Original languageEnglish (US)
Pages (from-to)438-444
Number of pages7
JournalThe Neurohospitalist
Volume13
Issue number4
DOIs
StatePublished - Oct 2023

Keywords

  • acute disseminated < autoimmune diseases of the nervous system
  • autoimmune diseases of the nervous system
  • cranial nerve diseases
  • encephalomyelitis
  • optic neuritis < cranial nerve diseases
  • seizures < epilepsy

ASJC Scopus subject areas

  • Clinical Neurology

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