Other Autoimmune CNS Demyelinating Disorders

Published on 03/03/2015 by admin

Filed under Neurology

Last modified 03/03/2015

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47 Other Autoimmune CNS Demyelinating Disorders

There are three very rare acute demyelinating central nervous system disorders that are distinct from multiple sclerosis (MS). These initially appear de novo in previously healthy individuals. Although some instances may occur in isolation, and respond to very high dose immunosuppressive therapy, on other occasions their consequences can be devastating.

Neuromyelitis Optica / Devic Disease

Optic neuritis (ON) is very commonly the first clinical event experienced by patients who later develop full-blown multiple sclerosis. Approximately 50% of ON patients will demonstrate clinical evidence of MS within 5–6 years of onset. Rarely an occasional patient, presenting with what seems to be classic ON, very soon thereafter develops an acute and clinically severe myelopathy. The combination represents an entirely different nosologic and pathophysiologic entity from MS. This entity, known as neuromyelitis optica (NMO), is a syndrome that is both relapsing and much more rapidly disabling than occurs in the typical MS patient. For example, about half of NMO patients become wheel chair bound within 5 years and almost two thirds will be legally blind (no better than 20/200) within that same time frame. There is no chronic progressive stage as most often eventually occurs in classic multiple sclerosis. NMO also has a relatively high early mortality, something almost unheard of in MS. These relatively rare NMO deaths usually follow disease extension into the brainstem and/or upper cervical cord.

Treatment

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