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It is possible that the main title of the report Neuromyelitis Optica is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
- Optic Neuroencephalomyelopathy
- Optic Neuromyelitis
- Retrobulbar Neuropathy
- Devic disease
- Devic syndrome
Related Disorders List
Information on the following diseases can be found in the Related Disorders section of this report:
Neuromyelitis optica, also known as Devic disease (DD), is a chronic disorder of nerve tissue characterized by inflammation of the optic nerve (optic neuritis) and inflammation of the spinal cord (myelitis). There appear to be two forms of this disease. In the classical, but less common type, there is a series of attacks over a short period of time (days or weeks) but, after the initial outburst, there are seldom repeat incidents. The second form is more common and is characterized by repeated attacks separated by periods of remission. In this form, the interval between attacks may be weeks, months or years. In its early stages, Devic disease may be confused with multiple sclerosis.
Neuromyelitis optica is initially marked by a slight fever, sore throat, and/or head cold. The characteristic symptoms are either optic neuritis or myelitis; either may occur as the first symptom. Optic neuritis is inflammation, of the optic nerve (optic neuritis) leading to pain inside the eye which rapidly is followed by loss of clear vision (acuity). Usually, only one eye is affected (unilateral) although both eyes may be involved simultaneously (bilateral).
The other cardinal syndrome is inflammation of the spinal cord, a condition known as transverse myelitis because the symptoms tend to affect all motor, sensory and autonomic functions (bladder and bowel) below a certain level on the body. Affected individuals may experience pain in the spine or limbs, and mild to severe paralysis (paraparesis to paraplegia) of the lower limbs, and loss of bowel and bladder control. Deep tendon reflexes may be diminished or absent initially, and later become exaggerated. A variable degree of sensory loss may occur. Affected individuals may also have a stiff neck, back pain, and/or headaches. This syndrome may be indistinguishable from other cases of "idiopathic" transverse myelitis.
Early in the course of the disease, it may be difficult to distinguish between Devic disease and multiple sclerosis because both may cause optic neuritis and myelitis as symptoms. However, the optic neuritis and myelitis tend to be more severe in neuromyelitis optica; the brain MRI is more commonly normal, and the spinal fluid analysis does not usually show oligoclonal bands in neuromyelitis optica, which are features that help distinguish it from MS.
In most cases of neuromyelitis optica, the initial symptoms of vision loss or paralysis improve with standard treatment with high dose corticosteroids, and partial recovery of vision, motor, sensory, or bladder function occurs. However, in recurring cases, neuromyelitis optica frequently causes significant permanent disturbances of vision and/or spinal cord function leading to blindness or impaired mobility.
Some patients with neuromyelitis optica have only recurrent myelitis or only recurrent optic neuritis. When patients have antibodies to aquaporin-4 with just these manifestations, many investigators would argue that they should be treated as having neuromyelitis optica. Brain lesions may occur in patients with neuromyelitis optica, typically, but not always, in later phases of the disease. Intractable vomiting or hiccups is now a generally accepted specific syndrome of this condition and is the result of inflammation in the dorsal medulla of the brainstem. Brainstem and hypothalamic syndromes are particularly common, but inflammation of the forebrain may also occur, often associated with prominent brain swelling (edema). Clinicians suspecting this disorder must have a strong index of suspicion for this condition especially in patients with a history of severe myelitis or optic neuritis.
Greater than 95% of patients with neuromyelitis optica report no relatives with the disease, but a small number of familial cases have been described. There is a strong association with a personal or family history of autoimmunity, which are present in 50% of cases. Neuromyelitis optica is regarded as an autoimmune disease though the exact cause for the autoimmunity is unknown.
Autoimmune disorders occur when the body's natural defenses against disease or invading organisms (such as bacteria), for unknown reasons, suddenly begin to attack healthy tissue. These defenses, for reasons not at all understood, attack proteins in the central nervous system, especially aquaporin-4.
Neuromyelitis optica occurs in individuals of all races. Relative to MS that it mimics, it occurs with greater frequency in individuals of Asian descent and possibly in individuals of African descent, but the majority of patients with this illness in Western countries are Caucasian. Individuals of any age may be affected, but typically Devic disease occurs in late middle-aged women. Equal numbers of men and women have the form that does not recur after the initial flurry of attacks, but women are four or five times more likely to be affected than men by the recurring (relapsing) form. Children represent another subset of patients affected by this condition; they more commonly develop brain symptoms at onset.
Symptoms of the following disorders can be similar to those of Neuromyelitis optica. Comparisons may be useful for a differential diagnosis:
Guillain-Barre syndrome, also known as acute idiopathic polyneuritis, is an autoimmune disorder that occurs when the body's defense system attacks the nerves, damaging the nerve's fatty sheath (myelin) and axis cylinder (axon). Nerve signals are delayed and altered, causing weakness and paralysis of the muscles of the legs, arms, and other parts of the body along with abnormal sensations. Fischer's syndrome (a form of polyneuroradiculitis marked by ophthalmoplegia, ataxia and arreflexia) and chronic idiopathic polyneuritis (chronic inflammation of groups of spinal nerve cells with an unknown origin) are two very rare forms of the disorder. (For more information on this disorder, choose "Guillain-Barre" as your search term in the Rare Disease Database.)
Acute disseminated encephalomyelitis (postinfectious encephalitis) is a central nervous system disorder characterized by inflammation of the brain and spinal cord caused by damage to the fatty sheath surrounding the nerves. This can occur spontaneously, but usually follows a viral infection or inoculation such as a bacterial or viral vaccine.
Multiple sclerosis is a chronic disease of the brain and spinal cord (central nervous system) which may be progressive, relapsing and remitting, or stable. The pathology of MS consists of small lesions called plaques that form randomly throughout the brain and spinal cord. These plaques are due to loss of the fatty sheath surrounding nerves and prevents proper transmission of nervous system signals and thus result in a variety of neurological symptoms. Most individuals with MS have a near normal life span. Symptoms often include visual difficulties as well as speech impairment, abnormal skin sensations or numbness, gait disturbance, and difficulties with bladder and bowel function. In a small number of cases, Devic disease has occurred as a complication of MS. (For more information on this disorder, choose "Multiple Sclerosis" as your search term in the Rare Disease Database.)
Systemic lupus erythematosus (also known as lupus) is an inflammatory connective tissue disease that can affect many parts of the body including the joints, skin and internal organs. Lupus is a disease of the body's immune system, most often striking young women between the ages of fifteen and thirty-five years. (For more information on this disorder, choose "Lupus" as your search term in the Rare Disease Database.)
A diagnosis of Devic disease is made based upon a detailed patient history, a thorough clinical evaluation, identification of characteristic physical findings, and a variety of specialized tests. Such tests include blood tests, examination of cerebrospinal fluid (CSF), spinal taps, or x-ray procedures such as magnetic resonance imaging (MRIs) or computed tomography (CT or CAT) scans. A blood test, NMO-IgG, has been recently discovered which is highly specific and moderately sensitive for neuromyelitis optica. It has been shown that it detects antibodies that are specific for an astrocyte protein, aquaporin-4. This is very helpful to request at the first significant suspicion of neuromyelitis optica, and is frequently positive at the time of the very first symptom even before a confident clinical diagnosis is possible. Successful diagnosis of Devic disease depends on distinguishing it from MS.
For acute attacks, the standard treatment is high-dose intravenous corticosteroids, typically methylprednisolone. Plasma exchange may be effective in patients who experience acute severe attacks that do not response to intravenous corticosteroids. This procedure involves removing some blood and mechanically separating the blood cells from the fluid (plasma). Blood cells are then mixed with a replacement solution and returned to the body.
For long-term suppression of the disease, no specific treatment has been proven, but azathioprine is regarded by many clinicians as first-line therapy. Other immunosuppressive agents, including mycophenolate mofetil and mitoxantrone may also be effective. Recently, rituximab has been shown to be helpful in two retrospective studies, including in patients who fail first-line immunosuppressive treatments. No clinical trials of long term treatments have been conducted yet in patients with neuromyelitis optica.
Symptom treatment may also involve the use of low doses of carbamazepine to control paroxysmal (sudden) tonic spasms that often occur during attacks of neuromyelitis optica and antispasticity agents to treat long term complication of spasticity that frequently develops in those with permanent motor deficits.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
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FROM THE INTERNET
More about Devic's Disease. Mayo Clinic. ©2006. 2pp.
Devic's Disease. Transverse Myelitis Association. Last Modified: 11-May-2006. 3pp.
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