Devic's Syndrome (Neuromyelitis Optica)

What is Devic's Syndrome?

Devic's syndrome is a rare autoimmune central nervous system disorder characterised by transverse myelitis (in which the fatty, protective covering of the spinal cord breaks down) and optic neuritis (in which inflammation of the optic nerve causes loss of vision and eye pain).]. It is considered a special form of multiple sclerosis (MS) with a severe and rapid course. The disorder affects the optic nerve and the nerves in the spinal cord. In Devic's syndrome, the fatty sheath that protects these nerves is lost. Individuals may experience vision impairment and various degrees of paralysis, as well as incontinence. The disorder is closely linked with MS and lupus, but usually appears before any symptoms of MS are noted. If an isolated disease episode affecting the spinal cord and optic nerve occurs after an infection or common cold, it is considered a post-infectious acute demyelinated encephalomyelitis (ADE) rather than Devic's syndrome.

Is there any treatment?

There is currently no standard treatment for Devic's syndrome. Generally, treatment is symptomatic and supportive. Corticosteroids may be prescribed. Treatment for ADE may include corticosteroids, intravenous immunoglobulin, and intravenous methylprednisolone

What is the prognosis?

Devic's syndrome is fatal in many patients. Some ADE patients achieve complete or nearly complete recovery while others may have residual deficits. Some severe cases of ADE may be fatal.