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Devic's disease, also known as neuromyelitis optica or NMO, is the simultaneous inflammation of the optic nerve (optic neuritis) and the spinal cord (myelitis).
Discovery
In 1870, Sir Thomas Clifford Allbutt first reported an association between myelitis and an optic nerve disorder. In 1894, Eugène Devic and his student Gault described 16 patients who had lost vision in one or both eyes and within weeks developed severe spastic weakness of the limbs, loss of sensation and often, bladder control. They recognized this was the result of inflammation of the optic nerve and of spinal cord respectively.
Similar instances of optic neuritis and myelitis were reported and many believed it constituted a distinct clinical entity. However some had pathology in other parts of the brain which was more suggestive of acute disseminated encephalomyelitis (ADEM), or multiple sclerosis(MS).
Symptoms
About one third of cases have preliminary symptoms of fever, myalgia, or headache. The typical patient has an acute and severe spastic weakness of the legs (paraparesis) or all four limbs (tetraparesis) with sensory signs, often accompanied by loss of bladder control. The disease develops over about one to fourteen days. Neuroimaging (MRI) excludes spinal cord compression. This weakness is preceded or succeeded by an acute unilateral or bilateral optic neuropathy with impaired visual acuity and colour vision and central hazy or opaque patches of loss of vision, but there are no signs beyond the spinal cord or optic nerves. Some measure of improvement in a few weeks is the rule, but residual signs and disability may persist, sometimes severely.
The disease can be a single episode, or recur with relapses and remissions. In about 80% cases, involvement of the cord and optic nerves occur within 3 months of each other.
Treatment
Treatment with short courses of high dosage intravenous steroids (methylprednisolone), plasma exchanges, or immunoglobulins may help to control the acute symptoms, but many trials contain very small numbers, and most are uncontrolled. Some recover, but many are left with impairment of vision and limbs, which can be severe.
Whether NMO is a distinct disease or part of the wide spectrum of multiple sclerosis is debated. Differences are the severe sequelae after an acute episode, which are more frequent in neuromyelitis optica than in MS. Multiple sclerosis infrequently presents as transverse myelitis. Oligoclonal bands in the CSF, aswell as white matter lesions on brain MRI are uncommon in neuromyelitis optica but occur in over 90% of MS patients.
NMO has been associated with many systemic diseases: collagen vascular diseases, autoantibody syndromes, infections - Varicella-zoster virus, Epstein-Barr virus, and HIV - and exposure to Clioquinol and Antituberculous drugs. These should be investigated when clinical features are suggestive.
Neuromyelitis optica emerges as a syndrome rather than a single disease. Separation from classic and variants of multiple sclerosis and variant forms of disseminated encephalomyelitis has been widely proposed, but no certain diagnostic features have been proved. Many sufferers are left with significant disabilities, requiring supportive and symptomatic treatment.
External links
- Devic's Disease (Neuromyelitis Optica)
- Mayo Clinic: Devics Disease - Information and treatment options
- The Walton Centre for Neurology and Neurosurgery NHS Trust, Liverpool, UK- Information for Patients, Doctors and Researchers
"Devic's disease"