Anti-myelin Oligodendrocyte Glycoprotein Antibody-Associated Disorder (MOGAD) in a Pediatric Patient with Rare Presentation of a Cerebellar Tumefactive Lesion

Objective NA.

Background Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies have been commonly associated with optic neuritis, myelitis, and acute disseminated encephalomyelitis but rarely with tumefactive lesions, especially in children. We report a young child with MOGAD presenting with a tumefactive cerebellar demyelinating lesion.

Design/Methods A retrospective chart review

Results A 3-year-old developmentally appropriate boy with fever for five days prior presented for gait changes and a self-resolved seizure lasting less than 5 minutes. Neurologic examination showed abnormal finger to nose on the left side, weakness of the left lower extremity and an ataxic gait. The differential at the time was Todd's paralysis versus an intracranial process. MRI showed a non-enhancing, ill-defined T2 hyperintense tumefactive lesion with mass effect within the left cerebellum concerning for tumor, abscess, or demyelination. On EEG, a lack of a well sustained and modulated posterior dominant rhythm and lack of a well-developed anterior to posterior gradient, with moderate background slowing was seen. Cerebrospinal fluid showed 8 white blood cells, 0 red blood cells, 55 mg/dl glucose, 31 mg/dl protein, 0.61 IgG index, and 0 oligoclonal bands. The Mayo Clinic cell-based assay detected anti-MOG IgG antibody in the serum with titer of 1:100. Neurological symptoms gradually improved after steroid pulse therapy.

Conclusions This report highlights the novel spectrum of radiologic manifestations associated with MOGAD in pediatric patients and MOGAD should always be considered in the differential diagnosis of tumefactive lesions.