Abstract

Myelin oligodendrocyte glycoprotein (MOG) antibodies have been recently reported in recurrent optic neuritis, aquaporin-4 antibody negative neuromyelitis optica and acute disseminating encephalomyelitis. Objective To describe the MOG antibody status, clinical characteristics and neuroradiological findings in children with multiphasic demyelinating encephalomyelitis (MDEM). Methods Clinical course, cerebrospinal fluid-, MRI studies and outcome of children with MDEM and serum MOG antibody status referred from different hospitals were reviewed. Results 9 children with two or more episodes (range 2 to 6) fulfilling the IPMSSG criteria of ADEM and serum MOG antibodies were identified. Median age at initial presentation was 4 years (range: 2 to 8 years). Median CSF cell count was 9/μl (range: 0 to 85/μl). OCBs tested in 7/9 children were present only in one child. Serum samples were available from all children from at least one acute episode. Median serum MOG antibody level was 1:1280 (range from 1:160 to 1:10240). One or more follow-up samples were available from 5 children showing persisting MOG antibodies overtime in all samples. Clinical outcome did range from normal (2/9), mild/moderate impairment with neuropsychological or motor deficits (3/9) to significant neurological sequelae (2/9). MR-imaging showed in general multiple hazy and large lesions which resolved or diminished in size before the next episode of ADEM occurred. In children with a moderate or severe neurological sequelae follow-up MRIs revealed large confluent lesions or generalized atrophy. Conclusion A subgroup of children with MDEM has high and persisting MOG antibodies extending the spectrum of MOG antibody associated diseases.

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