Abstract
Myelin oligodendrocyte glycoprotein (MOG) antibody disease (MOG-AD) is now recognised as a nosological entity with specific clinical and paraclinical features to aid early diagnosis. Although no age group is exempt, median age of onset is within the fourth decade of life, with optic neuritis being the most frequent presenting phenotype. Disease course can be either monophasic or relapsing, with subsequent relapses most commonly involving the optic nerve. Residual disability develops in 50–80% of patients, with transverse myelitis at onset being the most significant predictor of long-term outcome. Recent advances in MOG antibody testing offer improved sensitivity and specificity. To avoid misdiagnosis, MOG antibody testing should be undertaken in selected cases presenting clinical and paraclinical features that are felt to be in keeping with MOG-AD, using a validated cell-based assay. MRI characteristics can help in differentiating MOG-AD from other neuroinflammatory disorders, including multiple sclerosis and neuromyelitis optica. Cerebrospinal fluid oligoclonal bands are uncommon. Randomised control trials are limited, but observational open-label experience suggests a role for high-dose steroids and plasma exchange in the treatment of acute attacks, and for immunosuppressive therapies, such as steroids, oral immunosuppressants and rituximab as maintenance treatment.
Highlights
Myelin oligodendrocyte glycoprotein (MOG) is a glycoprotein located on the myelin surface and found exclusively in the central nervous system (CNS) [1, 2]
We review the clinical features, investigations and challenges of managing patients with MOG antibody disease (MOG-AD)
[44] Commonly < 50 WCC/mm3; OCBs in up to 95% of MOG-AD myelin oligodendrocyte glycoprotein antibody disease, NMO neuromyelitis optica, MS multiple sclerosis, Optic neuritis (ON) optic neuritis, ADEM acute disseminated encephalomyelitis, LETM longitudinally extensive transverse myelitis, Magnetic resonance imaging (MRI) magnetic resonance imaging, CSF cerebrospinal fluid, OCBs oligoclonal bands, WCCwhite cell count, RRMS relapsing–remitting multiple sclerosis be restricted to selected cases only, whereby the clinical and paraclinical features are felt to be in keeping with MOG-AD [16]
Summary
Myelin oligodendrocyte glycoprotein (MOG) is a glycoprotein located on the myelin surface and found exclusively in the central nervous system (CNS) [1, 2]. Peripheral demyelination, as MOG is not expressed in the peripheral nervous system; a MRI lesion adjacent to the lateral ventricle that is ovoid/round or associated with an inferior temporal lobe lesion, or Dawson’s finger-type lesions; an active brain MRI over time with silent increase in lesion burden between relapses; bi- or tri-specific measles, rubella and zoster virus reaction in the CSF; serum MOG-IgG levels at or just barely above the assay-specific cut-off, especially if the clinical picture is atypical; positive MOG-IgM and/or MOG-IgA result with negative MOG-IgG; MOG-IgG positivity in the CSF, but not in the serum, given that MOG-IgG is typically produced extra-thecally; AQP4-IgG/MOG-IgG “double-positive” test results, which are very rare and should prompt re-testing for both antibodies [16] In all these cases, caution should be exerted in diagnosing MOG-AD and clinical and paraclinical follow-up is advised.
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