MOG-IgG-associated demyelination: focus on atypical features, brain histopathology and concomitant autoimmunity
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Antibodies to myelin oligodendrocyte glycoprotein (MOG) have been demonstrated in patients with optic neuritis (ON), encephalitis and myelitis.
To describe the clinical and paraclinical features in patients with MOG-associated demyelination, focusing on unusual cases, brain biopsy and concomitant autoimmunity.
A single centre retrospective observational case series, analysing demographic, clinical, laboratory, histopathology and radiological data from MOG- positive patients.
We identified 20 adults. The male/female ratio was 1.5. Mean age at onset was 31.6 years and mean disease duration was 7.5 years. The most frequent presentation was myelitis (45%), followed by ON (30%). One case had simultaneous myelitis and ON. Two patients had a cortical syndrome, 1 patient had an encephalopathic presentation and 1 cryptogenic focal epilepsy. Anti-neutrophil cytoplasmic antibodies (ANCA) were found in 3 cases, while 1 patient had an antibody to glutamic acid decarboxylase (GAD). Brain biopsy was performed in 2 patients. Relapsing course was identified in 60% of patients. We also discuss 3 cases with atypical features, brain histopathology and concomitant autoimmunity.
MOG- associated demyelination represents a new disease entity. Unusual cases are reported, expanding the disease spectrum. Elucidating this further should be the focus of prospective studies.
KeywordsMOG Myelin oligodendrocyte glycoprotein Concomitant autoimmunity Histopathology
We thank the patients who gave consent for us to report their individual cases. We thank Dr N. Evangelou, Dr C. Gilmore and Dr B. Gran, who provided patients for this study. We thank Dr G. Sare for critically reading the manuscript and useful comments.
This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Compliance with ethical standards
Conflicts of interest
The authors declare that there is no conflict of interest.
The study was approved by the Quality and Safety Committee of Nottingham University Hospitals NHS Trust.
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