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

Area of Science:

• Neuroimmunology
• Pediatric Neurology

Background:

• Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory demyelinating disorder.
• Understanding its diverse clinical presentations is crucial for timely diagnosis and management.

Observation:

• Two pediatric cases of MOGAD with challenging initial presentations are detailed.
• Case 1: A 12-year-old boy with refractory headaches, cranial neuropathies, and intracranial hypertension.
• Case 2: A 3-year-old boy with new-onset refractory seizures (FLAMES variant).

Findings:

• Both patients exhibited cortical T2 hyperintensities, leptomeningeal enhancement, and bilateral optic nerve enhancement on MRI.
• Cerebrospinal fluid analysis revealed pleocytosis with neutrophilic predominance in both cases.
• Treatment involved immunotherapies including IVIg, plasma exchange, and corticosteroids, with rituximab added for the second patient.

Implications:

• These cases underscore the pleomorphic clinical phenotypes of MOGAD in children.
• Highlights the importance of considering MOGAD in pediatric patients with unexplained neurological symptoms.
• Demonstrates variable treatment responses, necessitating personalized therapeutic strategies.