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What’s new in neuromyelitis optica? A short review for the clinical neurologist.

J Neurol. 2017 Mar 13. doi: 10.1007/s00415-017-8445-8. [Epub ahead of print]

Whittam D, Wilson M, Hamid S, Keir G, Bhojak M, Jacob A.

The evolution of neuromyelitis optica spectrum disorder (NMOSD) from a rare, incurable and misunderstood disease with almost universally poor outcomes to its present state in just over a decade is unprecedented in neurology and possibly in medicine. Our knowledge of NMOSD biology has led to the recognition of wider phenotypes, new disease mechanisms, and thus clinical trials of new and effective treatments. This article aims to update readers on the recent developments in NMOSD with particular emphasis on clinical advances, the 2015 diagnostic criteria, biomarkers, imaging, and therapeutic interventions.

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Introduction

Neuromyelitis optica spectrum disorder (NMOSD) is a relapsing and often severely disabling autoimmune disease, which predominantly targets the spinal cord, optic nerves and brainstem. Three quarters of patients with NMOSD have serum immunoglobulin-G (IgG) autoantibodies to the aquaporin-4 channel (AQP4-IgG), which is highly expressed on the ‘foot-processes’ of astrocytes at the blood–brain barrier [1, 2]. 

Anti-myelin-oligodendrocyte glycoprotein antibody (MOG-IgG)

A proportion of patients with AQP4-IgG seronegative NMOSD and NMOSD-like syndromes (e.g. relapsing optic neuritis or relapsing transverse myelitis) have been found to have serum antibodies against myelin-oligodendrocyte glycoprotein (MOG-IgG). In contrast to AQP4-IgG seropositive NMOSD, which is an astrocytopathy, MOG-IgG targets a protein expressed by oligodendrocytes and on the outermost surface of myelin sheaths [37, 38] (see Fig. 1). MOG-IgG has also been linked with other demyelinating diseases, including paediatric forms of multiple sclerosis and acute disseminated encephalomyelitis (ADEM) [37, 39, 40]. Whether these apparent MS cases are truly the same as adult relapsing MS will be borne out only on long-term follow-up. Access full article >

 

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