Rare Presentation of Myelin Oligodendrocyte Glycoprotein Antibody-associated Disease in Four Children

Abstract Background: Myelin oligodendrocyte glycoprotein antibody associated disease (MOG-AD) is an inflammatory disorder of the central nervous system, identified more frequently in children than adults. We report 4 children with MOG-AD. Clinical Description: Cases 1–3 presented with fever, headache, and vomiting for varying durations. Case 4 presented with acute-onset impaired vision, after a prior episode of meningoencephalitis-like picture. Examination showed that Cases 1 and 2 had neck rigidity, and Case 4 had papilledema along with reduced visual acuity. Other cranial nerves, power, tone, and reflexes were normal in all children. Management and Outcome: Laboratory investigations were largely normal except elevated total leukocyte count and C-reactive protein in Cases 1 and 3 with a marginal rise in C- reactive protein in Cases 2 and 4. Cerebrospinal analysis showed pleocytosis with lymphocytic predominance and increased protein in Cases 3 and 4. Magnetic resonance imaging revealed features consistent with meningoencephalitis in Cases 1 and 3, acute disseminated encephalomyelitis in Case 2, and optic neuritis in Case 4. All the children had positive titers of anti-MOG, thus being diagnosed as MOG-AD. All the children were treated with intravenous methylprednisolone with a few of them requiring additional immunomodulator therapy. All the cases showed excellent recovery and remained in remission till the last follow-up. Conclusion: Children presenting with features simulating meningoencephalitis may be due to the rare condition of MOG-AD. A knowledge about this condition and specific investigations with expert interpretation and specific treatment can result in favorable outcomes.