Clinical features of the first attack with leukodystrophy-like phenotype in children with myelin oligodendrocyte glycoprotein antibody-associated disorders.

Background: Myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) is identified autoimmune disorder with a predominance in paediatric patients, and the disease spectrum has expanded with clinical and radiological patterns. The aim of the study was to describe the clinical characteristics of the first attack with leukodystrophy-like phenotype with MOGAD in children.

Methods: Patients hospitalized at the Children's Hospital of Chongqing Medical University from June 2017 to October 2021 with positive MOG antibodies and phenotype of leukodystrophy-like (symmetric white matter lesions) were retrospectively analyzed. Cell-based assays (CBAs) were used to test MOG antibodies.

Results: Four cases from 143 MOGAD patients were recruited, with two females and two males. The age of onset is all under 6 years old. At the last follow-up, four cases exhibited a monophasic course, including ADEM in three patients and encephalitis in one patient. The mean EDSS score at onset was 4.62 ± 2.93, and the modified Rankin score (mRS) was 3.00 ± 1.82. First-attack symptoms include fever, headache, vomiting, seizure, loss of consciousness, emotional and behavioural disorder, and ataxia. The brain MRI showed prominent extensive and essentially symmetric distribution lesions in the white matter. All patients showed clinical and partial radiological improvement after intravenous immunoglobulin and/or glucocorticoid treatment.

Conclusion: The first attack with MOGAD onset of leukodystrophy-like phenotype was more frequently seen in younger children than other phenotype patients. The patients may show impressive neurologic disorders, but most patients who receive immunotherapy have a good prognosis.