Clinical characteristics of myelin oligodendrocyte glycoprotein antibody neuromyelitis optica spectrum disorder

Abstract

Background.-Serological antibodies against myelin oligodendrocyte glycoprotein (MOG) are associated with a relapsing autoimmune demyelinating disease of the central nervous system. Initially identified in the context of acute disseminated encephalomyelitis, persistent seropositivity of MOG antibodies is now recognized as a variant of neuromyelitis optica spectrum disorder (NMOSD). Objectives:The aim of the study is to describe the epidemiological and clinical features of MOG antibody positive cases and compare our findings with those previously published.Methods.-This is a retrospective descriptive study of 23 patients with MOG antibody disease who were cared for at Johns Hopkins Hospital over the period from 2015 to 2018. MOG testing was done at Johns Hopkins using the cell based assay (CBA). We describe their epidemiological and clinical features.Results.-Twenty-three patients were included in the study with a female to male ratio of 2.3:1. The mean age of the cohort was 42.6 years, while the mean age at onset was 37 years. The most frequent initial presentation was optic neuritis, followed by ADEM-like encephalopathic clinical picture and transverse myelitis. Five patients showed a monophasic disease course while the rest experienced a relapsing phenotype. Nine patients (39%) experienced immediate relapses on withdrawal of steroids.conclusions.-Our cohort showed clinical characteristics comparable with previously published reports of MOG antibody disease worldwide. Unique features of MOG antibody disease are: high frequency of optic neuritis attacks, good long term neurological recovery and sensitivity to steroid use and withdrawal.

Clinical analysis of a patient simultaneously positive for antibodies of myelin oligodendrocyte glycoprotein and anti–N-methyl-D-aspartate receptor

“…Unique features of MOG-AD are sensitivity to steroid use and withdrawal. [ 7 ] The patient showed good response to a small amount of steroids, consistent with the diagnosis of MOG-AD. [ 14 ] During follow-up (a minimum of 6 months), 58 (52.7%) patients with MOG-optic neuritis (MOG-ON) experienced at least one episode of recurrence of ON.…”

“…Our case met the diagnostic criteria of MOG-AD, [ 7 ] but not of the typical anti-NMDARe. [ 8 ] The clinical findings were inconsistent with the diagnosis of overlapping MOG-AD and NMDARe.…”

“…Unique features of MOG-AD are the high frequency of ON attacks, good long-term neurological recovery, and sensitivity to steroid therapy. [ 7 ] There is a clear distinction in the clinical characteristics (including main symptoms, MRI findings, and response to steroid treatment) between MOG-AD and anti-NMDARe. Diagnosis of the attack in terms of its association with the two diseases plays an important role in the treatment decision and prognostication.…”

E.U. paediatric MOG consortium consensus: Part 1 – Classification of clinical phenotypes of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders

“…Recently, this association between MOG-abs and encephalitis has been explored further in two paediatric studies [24,103], including one large prospective, multicentre, observational paediatric cohort study from Spain [23]. In this study, among the 296 patients with definite or possible encephalitis (according to international criteria [103]), 7% had MOG-abs [23].…”

“…Furthermore, 85% of these patients presented with neurological bowel and bladder dysfunction, and additionally 54% of male patients with erectile dysfunction, probably attributable to the often longitudinally extensive lesion with frequent conus involvement observed on MRI in these patients (41%). This conus involvement has also been demonstrated in 37% of all paediatric MOG-ab-positive patients presenting with spinal cord lesions [24]. While LETM is also characteristic for AQP4-ab-associated TM, conus involvement is more typical for MOG-TM [33,60e62].…”