Clinical features of recurrent MOG antibody-associated cortical encephalitis in adults

Objective: To clarify the clinical features of recurrent myelin oligodendrocyte glycoprotein antibody-associated cortical encephalitis (MOGCE) in adults.

Methods: We present an adult case of recurrent MOGCE and summarize the clinical symptoms, imaging findings, treatment and prognosis of this phenotype as per a systematic review of the literature.

Results: We identified 9 adult patients with recurrent MOGCE. The mean age was 32 years, and 5/9 were male. Median time to recurrence was 6 months (range 2-36 months). The most common presentations of the first attack were headache (9/9), fever (8/9) and seizure (5/9). In most patients (6/9), presentations of recurrent attacks were different than those of the first attack. Relapses may affect more areas than the first attack, such as spine, brainstem, grey matter, and basal ganglia. All patients were seropositive for MOG antibodies during the first or second attack, with antibody titers ranging from 1:10 to 1:100. CSF white blood cell count and total protein were elevated in 6/9 patients. On MRI, 4/9 showed bilateral FLAIR hyperintense lesions, while 5/9 had unilateral lesions. Most patients demonstrated a positive response to treatment, and maintenance immunotherapy was added upon relapse.

Conclusion: The clinical presentation of recurrent MOGCE is atypical, and most patients had different symptoms upon recurrence compared to the first episode. Factors contributing to the likelihood of disease recurrence remain unclear. Most recurrent MOGCE patients respond well to immunotherapy, and require long-term immunotherapy after recurrence.