'Leukodystrophy-like' phenotype in children with myelin oligodendrocyte glycoprotein antibody-associated disease

Yael Hacohen, Thomas Rossor, Kshitij Mankad, Wk Kling Chong, Andrew Lux, Evangeline Wassmer, Ming Lim, Frederik Barkhof, Olga Ciccarelli, Cheryl Hemingway

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76 Citations (Scopus)


Aim: To review the demographics and clinical and paraclinical parameters of children with myelin oligodendrocyte glycoprotein (MOG) antibody-associated relapsing disease. Method: In this UK-based, multicentre study, 31 children with MOG antibody-associated relapsing disease were studied retrospectively. Results: Of the 31 children studied, 14 presented with acute disseminated encephalomyelitis (ADEM); they were younger (mean 4.1y) than the remainder (mean 8.5y) who presented with optic neuritis and/or transverse myelitis (p<0.001). Similarly, children who had an abnormal brain magnetic resonance imaging (MRI) at onset (n=20) were younger than patients with normal MRI at onset (p=0.001) or at follow-up (p<0.001). 'Leukodystrophy-like' MRI patterns of confluent largely symmetrical lesions was seen during the course of the disease in 7 out of 14 children with a diagnosis of ADEM, and was only seen in children younger than 7 years of age. Their disability after a 3-year follow-up was mild to moderate, and most patients continued to relapse, despite disease-modifying treatments. Interpretation: MOG antibody should be tested in children presenting with relapsing neurological disorders associated with confluent, bilateral white matter changes, and distinct enhancement pattern. Children with MOG antibody-associated disease present with age-related differences in phenotypes, with a severe leukoencephalopathy phenotype in the very young and normal intracranial MRI in the older children. This finding suggests a susceptibility of the very young and myelinating brain to MOG antibody-mediated mechanisms of damage. What this paper adds: Myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination manifest with an age-related phenotype. Children with MOG antibody and 'leukodystrophy-like' imaging patterns tend to have poor response to second-line immunotherapy.

Original languageEnglish
Pages (from-to)417-423
JournalDevelopmental Medicine and Child Neurology
Issue number4
Publication statusPublished - Apr 2018

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