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(Synonyms: neuromyelitis optica, NMO, Devic's syndrome)

About Devic's Disease

Devic's disease is an inflammatory disease of the central nervous system in which there are episodes of inflammation and damage to the myelin (fatty, protective covering of nerves) that almost exclusively affect the optic (eye) nerves and spinal cord. It usually causes temporary blindness, occasionally permanent, in one or both eyes. It can also lead to varying degrees of weakness or paralysis in the legs or arms, loss of sensation, and/or bladder and bowel dysfunction from spinal cord damage.

Types

It appears as though there are two major types of Devic's disease. In the first type, optic neuritis, (inflammation of the optic nerve), and myelitis, (inflammation of the spinal cord), episodes tend to come very close together often within days or weeks, and there is no recurrence after the initial flurry of symptoms. In the second form, repeated episodes of optic neuritis and myelitis occur that are separated by months or years.

Differences from Multiple Sclerosis

In well established cases of Devic's disease, it is usually possible to accurately tell the difference between Devic's disease and MS. However, early in their course, it may be difficult to definitively separate these two conditions. However, there are some differences.

Devic's disease affects only the optic nerves and spinal cord, whereas MS affects the brain as well.
Attacks of Devic's disease tend to be more frequent and severe than in MS, though this is not always the case.
An MRI of the brain is typically normal in Devics disease, although this is not always the case; in MS the MRI of the brain typically shows many areas of inflammation.
An MRI of the spinal cord shows large extensive areas of inflammation of the spinal cord whereas in MS typically the areas are much smaller.
Spinal fluid studies tend not to show the typical elevation of antibodies detected in patients with MS, although occasional patients may show this abnormal pattern of antibodies.
Outcomes of Devic's Disease

The course of Devic's disease is highly variable. It largely depends on whether there is a tendency for relapses to occur after the initial flurry of symptoms that leads to the diagnosis.

In general, attacks of Devic's disease tend to be more frequent and severe than they are in MS. The major risk to patients is severe damage to the upper spinal cord, which can lead to inability to breathe on one's own. This may be fatal. However, some patients with Devic's disease seem to enter a long period of time where the disease remains stable. Devic's disease has not been studied in large enough populations to predict the outcome of individual cased with great certainty.

Complications of Devic's Disease

Permanent blindness may occur in one or both eyes. Permanent loss of strength or sensation in the arms or legs may occur. Inability to control the bowel or bladder function may also occur.

At any point in this disease, patients may develop sudden brief, repetitive spasms. These spasms may also occur in MS, but they are very common in Devic's disease. With these spasms, patients develop prolonged tightening of arms and legs that last for 15 seconds to 2 minutes. They may be painful and recur several times a day.

TREATMENT
Currently, there is no cure for Devic's disease, but symptoms can be treated. Some patients recover, but many are left with impairment of vision and limbs, which can be severe.


Attacks are treated with short courses of high dosage intravenous corticosteroids such as methylprednisolone IV. When attacks progress or do not respond to corticosteroid treatment, plasmapheresis can be an effective treatment. Clinical trials for these treatments contain very small numbers, and most are uncontrolled.

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