Devic's Syndrome

Devic's syndrome is a rare autoimmune central nervous system disorder. It is characterized by transverse myelitis. With this, the fatty, protective covering of the spinal cord breaks down. It also causes optic neuritis. With this, inflammation of the optic nerve causes loss of vision and eye pain. This syndrome is considered a special form of multiple sclerosis (MS). It has 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. An isolated disease episode affecting the spinal cord and optic nerve that occurs after an infection or common cold is not Devic's syndrome. Rather it is considered to be a post-infectious acute (sudden) demyelinated encephalomyelitis (ADE).

SYMPTOMS

Patients may have:

  • Vision impairment.

  • Various degrees of paralysis.

  • Incontinence.

The disorder is closely linked with MS and lupus. But it usually appears before any problems (symptoms) of MS are seen.

TREATMENT

There is currently no standard treatment for this syndrome. Generally, treatment is symptomatic and supportive. Corticosteroids may be prescribed. Treatment for ADE may include:

  • Corticosteroids.

  • Intravenous immunoglobulin.

  • Intravenous methylprednisolone.

PROGNOSIS

This syndrome is fatal in many patients. Some ADE patients achieve complete or nearly complete recovery. But others may have lasting deficits. Some severe cases of ADE may be fatal.

RESEARCH BEING DONE

The NINDS supports an extensive research program of basic studies. This is to increase understanding of how the nervous system works. Major goals of this research are to develop methods to:

  • Repair damaged nerves.

  • Restore full use and strength to injured areas.