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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Devic disease


Other Names for this Disease
  • Devic syndrome
  • Devic's neuromyelitis optica
  • Neuromyelitis optica
  • NMO
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Overview



What is Devic disease?

What causes Devic disease?

How might Devic disease be treated?

What is the prognosis for individuals with Devic disease?


What is Devic disease?

Devic disease, is an autoimmune disease affecting the spinal cord and optic nerves (the nerves that carry information regarding sight from the eye). In Devic disease the protective outer covering of the nerves (myelin) is lost. The syndrome can also damage nerve fibers and leave areas of broken-down tissue.[1]  Signs and symptoms worsen with time and include optic neuritistransverse myelitis, sensory impairment, and bladder and bowel dysfunction.[2] Currently there is no cure for Devic disease, but there are therapies to treat an attack while it is happening, to reduce symptoms, and to prevent relapses. In its early stages, Devic disease may be confused with multiple sclerosis.[3] 
Last updated: 10/13/2010

What causes Devic disease?

Most cases of Devic disease are idiopathic (meaning that the underlying mechanism that triggers the condition is unknown). Studies of affected cells and tissues have improved our understanding of the disease process. In Devic disease immune proteins (autoantibodies) attach themselves to specialized proteins in the spinal cord and optical nerve called "water channel proteins" (specifically aquaporin-4 or AQP4). The autoantibodies signal immune cells and proteins to attack resulting in damage to myelin and the breakdown of healthy nerves and tissues.[4]

There have been a few cases of Devic disease occurring in association with certain infectious conditions (e.g., syphilis, HIV, chlamydia, varicella, cytomegalovirus, and Epstein Barr virus). The nature of this association isn't clear. It is possible that certain infections may trigger Devic disease in individuals who are predisposed to the condition.[4]
Last updated: 10/13/2010

How might Devic disease be treated?

There is no cure for Devic disease, but there are therapies to treat an attack while it is happening, to reduce symptoms, and to prevent relapses. Doctors usually treat an initial attack of Devic disease with a combination of a corticosteroid drug to stop the attack, and an immunosuppressive drug for prevention of additional attacks. If frequent relapses occur, some individuals may need to continue a low dose of steroids for longer periods. Plasma exchange (plasmapheresis) is a technique that separates antibodies out of the blood stream and is used with people who do not respond to corticosteroid therapy. Pain, stiffness, muscle spasms, and bladder and bowel control problems can be managed with the appropriate medications and therapies. Individuals with major disability will require the combined efforts of occupational therapists, physiotherapists, and social services professionals to address their complex rehabilitation needs.[1] 

The Transverse Myelitis Association provides additional information on the treatment options available for Devic disease. http://myelitis.org/symptoms-conditions/neuromyelitis-optica/acute-treatments-nmo/
Last updated: 4/9/2014

What is the prognosis for individuals with Devic disease?

The onset of Devic disease varies from childhood to adulthood, with two peaks, one in childhood and the other in adults in their 40s. Most individuals with Devic disease have the relapsing form of the disease and experience clusters of attacks months or years apart, followed by partial recovery during periods of remission. Disability is cumulative, the result of each attack damaging new areas of myelin. Another form of Devic disease is characterized by a single, severe attack extending over a month or two, with little recurrance after the initial onset of symptoms. Some individuals are severely affected by Devic disease and can lose vision in both eyes and the use of their arms and legs. Most individuals experience a moderate degree of permanent limb weakness from myelitis. Muscle weakness can cause breathing difficulties and may require the use of artificial ventilation. The death of an individual with Devic disease is most often caused by respiratory complications from myelitis attacks.[1]

Last updated: 5/7/2010

References
  1. NINDS Neuromyelitis Optica Information Page. National Institute of Neurological Disorders and Stroke (NINDS). 2007; http://www.ninds.nih.gov/disorders/neuromyelitis_optica/neuromyelitis_optica.htm. Accessed 11/18/2009.
  2. Weinshenker BG. Neuromyelitis Optica. NORD Guide to Rare Disorders. Philadelphia PA: Lippincott Williams & Wilkins; 2003;
  3. Weinshenker B. Neuromyelitis Optica. National Organization for Rare Disorders (NORD). 2008; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Neuromyelitis%20Optica. Accessed 11/18/2009.
  4. Hazin H, Khan F, Bhatti MT. Neuromyeliits optica: Current concepts and prospects for future management. Current opinion in opthalmology. 2009;
  5. Calabresi PA. Multiple Sclerosis and Demyelinating Conditions of the Central Nervous System. In: Goldman. Cecil Medicine, 23rd edition. Phildelphia, PA: Saunders; 2007;