Devic's Disease

(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. In most cases, they respond very successfully to treatment with an anticonvulsant medication.

Links to additional information:

• Acute Therapies for the Neuroimmunologic Disorders
• Neuroimmunologic Disorders of the Central Nervous System
• Mayo Clinic: Devics Disease - Information and treatment options
• Walton Neurology Centre in Liverpool, UK
• The Guthy-Jackson Charitable Foundation is dedicated to funding basic science research to find answers that will lead to the prevention, clinical treatment programs and a potential cure for Neuromyelitis Optica (NMO) Spectrum Disease.
• MyDailyApple Devic's disease
• Mayo Clinic: New Antibody Test for Devic's Disease Discovered
• Devic’s Syndrome: Close to a Cure?
• Gayle's Devic's Syndrome and NMO Support Group
• Neuromyelitis Optica: It's Not MS, Doctors Stress
• Devic's Disease Message Forum
• Google Groups : Devic's support - support group for Devic's or NMO, TM patients, and their caregivers or friends
• Obstetric Issues - TM, ADEM, NMO

MS Frontiers 2007 was the largest MS society conference for professionals yet, providing collaboration between the MS Societies of the UK, US, Australia and Canada and bringing together experts from across the world to speak on MS Research. Dr Eli Silber is the consultant neurologist at King’s College Hospital in London. He spoke about ethnic differences and the part they play in MS and NMO An outstanding presentation was given by Brian Weinshenker, professor of neurology at the Mayo Clinic of Medicine in Minnesota in the US on his recent work on Neuromyelitis optica (NMO). Professor Alastair Compston, Head of the Department of Clinical Neurosciences at the University of Cambridge spoke of Neuromyelitus optica (NMO) an inflammatory demyelinating disorder of the central nervous system that most commonly targets the optic nerves and spinal cord selectively.
Neuromyelitis Optica (NMO) or Devic's Disease presentation from the 2006 Rare Neuroimmunologic Disorders Symposium held in Baltimore, MD. Dean Wingerchuk, MD, Mayo Clinic, Scottsdale, AZ Wingerchuk_NMO_Rare Neuroimm_062406_final Wingerchuk_NMO_Clinical Session_071906 View Video
Neuromyelitis Optica (NMO) or Devic's Disease presentation from the 2004 Rare Neuroimmunologic Disorders Symposium held in Baltimore, MD. Dean Wingerchuk, MD, Mayo Clinic, Scottsdale, AZ Handouts 1.8Mb View Video 31 mins
The following video presentations are from the 2001 TM symposium held in Baltimore, MD.
Devic's Disease (NMO) Dean M. Wingerchuk, MD Associate Professor of Neurology Mayo Clinic, Scottsdale Scottsdale, Arizona View Handouts 53KB View Video 39 minutes	Immunopathology of Neuromyelitis Optica/Devic's Disease Claudia F. Lucchinetti, MD Associate Professor of Neurology Department of Neurology Mayo Clinic Rochester, Minnesota View Handouts View Video 30 minutes