Several case-based series of seizures associated with MOG-AD have also been reported. In case-based series, residual disability develops in 50–80% of patients, with transverse myelitis at onset being the most significant predictor of long-term disability. The impact of relapses on disability is variable: some studies report no difference between monophasic and relapsing disease courses, whilst others report worsening disability associated with higher relapse frequency. MOG-positive ON is frequently bilateral and associated with optic nerve head swelling. Ī relapsing course has been reported in 44–83% of patients and more commonly involves the optic nerve. The most common presenting feature is optic neuritis (ON), occurring in 54–61% of patients, followed by myelitis, acute disseminated encephalomyelitis (ADEM) or an ADEM-like presentation (e.g., brainstem attack). MOG-AD can occur in all decades of life, with a slight predominance in women and with median age of onset in the early to mid-thirties. Whether MOG antibodies play a pathogenic role in all these conditions, or if they represent a bystander effect or epiphenomenon, remains unclear. Īlthough in most cases demyelination associated with MOG antibodies occurs without any apparent inciting or predisposing event/illness, it has been associated with demyelinating N-methyl- d-aspartate receptor encephalitis, post-infectious demyelination following herpes simplex virus, Borrelia and Epstein–Barr virus infections and, more rarely, with typical relapsing MS. MOG-AD is an inflammatory demyelinating condition of the CNS characterised by a monophasic or relapsing course of neurological dysfunction, which does not meet the typical criteria for MS or other known neuroinflammatory conditions and occurs in the presence of serum MOG antibodies detected using specific cell-based assays. In this article, we review the clinical features, investigations and challenges of managing patients with MOG-AD.Ĭlinical features of MOG antibody disease Therefore, MOG antibody disease (MOG-AD) is now recognised as a distinct nosological entity with specific management and therapeutic requirements. However, the more recent development of highly sensitive and specific methods for MOG antibody detection using cell-based assays, along with new diagnostic classification of similar neuroinflammatory conditions, has made it possible to identify a subset of patients with antibodies to MOG who express a clinical phenotype distinct from MS or from neuromyelitis optica (NMO). This hypothesis was later supported by their discovery in the sera and cerebrospinal fluid (CSF) of patients with multiple sclerosis (MS), using techniques such as enzyme-linked immunosorbent assay and Western blot. MOG antibodies have been extensively studied over the last 30 years, with some early experimental studies hypothesising a pathogenic role in CNS inflammatory diseases. Although its exact role remains unclear, it is thought to act as a cellular adhesive molecule, to be involved as a regulator of oligodendrocyte microtubule stability and to mediate complement cascade. Myelin oligodendrocyte glycoprotein (MOG) is a glycoprotein located on the myelin surface and found exclusively in the central nervous system (CNS). 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. Cerebrospinal fluid oligoclonal bands are uncommon. MRI characteristics can help in differentiating MOG-AD from other neuroinflammatory disorders, including multiple sclerosis and neuromyelitis optica. 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. Recent advances in MOG antibody testing offer improved sensitivity and specificity. Residual disability develops in 50–80% of patients, with transverse myelitis at onset being the most significant predictor of long-term outcome. Disease course can be either monophasic or relapsing, with subsequent relapses most commonly involving the optic nerve. 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. 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.
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