Malignant Intracranial Pressure Elevation in MOGAD Meningoencephalitis

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) was first proposed as a distinct demyelinating disorder about a decade ago; however, its entire range of phenotypes has yet to be established. We present a case of a 15-year-old boy with features of viral meningoencephalitis with refractory elevated intracranial pressure requiring decompression ultimately diagnosed with an atypical phenotype of MOGAD. After treatment with steroids, 7 rounds of plasmapheresis, intravenous immunoglobulin, and tocilizumab, followed by a second attack and maintenance therapy, he improved to near baseline. Our intention is to raise awareness about the spectrum of less common phenotypes like cerebral cortical encephalitis. We review other atypical MOGAD presentations for comparison. Patients presenting with meningoencephalitis and a negative infectious workup, particularly in the setting of elevated intracranial pressure, should be tested for myelin oligodendrocyte glycoprotein immunoglobulin G as prompt diagnosis and initiation of immunotherapy may improve outcomes and reduce the risk of relapse.