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Transverse myelitis


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What is transverse myelitis?

Transverse myelitis is a neurological disorder caused by inflammation across both sides of one level, or segment, of the spinal cord. The term myelitis refers to inflammation of the spinal cord; transverse describes the position of the inflammation, that is, across the width of the spinal cord. Attacks of inflammation can damage or destroy myelin, the fatty insulating substance that covers nerve cell fibers. This damage causes nervous system scars that interrupt communications between the nerves in the spinal cord and the rest of the body. [1] Transverse myelitis is part of a spectrum of neuroimmunologic diseases of the central nervous system. Other disorders in this spectrum include, Acute Disseminated Encephalomyelitis (ADEM), Optic Neuritis, and Neuromyelitis Optica (Devic's disease). [2]
Last updated: 8/15/2008

What symptoms are associated with transverse myelitis?

Transverse myelitis may be either acute (developing over hours to several days) or subacute (developing over 1 to 2 weeks). Initial symptoms usually include localized lower back pain, sudden paresthesias (abnormal sensations such as burning, tickling, pricking, or tingling) in the legs, sensory loss, and paraparesis (partial paralysis of the legs). Paraparesis often progresses to paraplegia (paralysis of the legs and lower part of the trunk). Urinary bladder and bowel dysfunction is common. Many patients also report experiencing muscle spasms, a general feeling of discomfort, headache, fever, and loss of appetite. Depending on which segment of the spinal cord is involved, some patients may experience respiratory problems as well. [1]

From this wide array of symptoms, four classic features of transverse myelitis emerge: [1][3]

Weakness of the legs and arms: Some patients report stumbling, dragging one foot or notice that both legs seem heavier than normal. Depending on the level of involvement within the spinal cord, coordination or strength in the hands and arms may also be affected. [3]

Pain: Up to half of those with transverse myelitis report pain as the first symptom of the disorder. It can be localized to the back, or appear as sharp, shooting pain that radiates down the legs, arms or around the torso. [3]

Sensory alteration: Loss of the ability to experience pain or temperature sensitivity is one of the most common sensory changes. Patients who are experiencing altered sensitivity usually report numbness, tingling, coldness or burning. Up to 80 percent of patients experience heightened sensitivity to touch. Some even report that wearing clothes or a light touch with a finger causes significant pain. [3]

Bowel and bladder dysfunction: Some patients report bowel or bladder dysfunction as their first symptom of transverse myelitis. This may mean an increased frequency or urge to urinate or defecate, incontinence, difficulty voiding, sensation of incomplete evacuation or constipation. [3]


Last updated: 8/15/2008

What causes transverse myelitis?

Researchers are uncertain of the exact causes of transverse myelitis. [1] The inflammation that causes such extensive damage to nerve fibers of the spinal cord may occur in isolation or in the setting of another illness. When it occurs without apparent underlying cause, it is referred to as idiopathic. [1][4] Transverse myelitis is idiopathic in about 60% of cases. [3]

The following illnesses or agents have been associated with transverse myelitis:

Infectious agents. Transverse myelitis often develops following viral infections. Infectious agents suspected of causing transverse myelitis include varicella zoster (the virus that causes chickenpox and shingles), herpes simplex, cytomegalovirus, Epstein-Barr, influenza, echovirus, human immunodeficiency virus (HIV), hepatitis A, measles, and rubella. Bacterial skin infections, middle-ear infections (otitis media), syphilis, Lyme disease, and Mycoplasma pneumoniae (bacterial pneumonia) have also been associated with the condition. [1] In some cases, the infectious agent may directly invade the spinal cord and produce symptoms of transverse myelitis. [5] In other post-infectious cases of transverse myelitis, immune system mechanisms, rather than active viral or bacterial infections, appear to play an important role in causing damage to spinal nerves. [1][5] Although researchers have not yet identified the precise mechanisms of spinal cord injury in these cases, stimulation of the immune system in response to infection indicates that an autoimmune reaction may be responsible. [1]

Autoimmune diseases. Transverse myelitis occasionally develops in people who have other autoimmune diseases. [5] These may include systemic lupus erythematosus, Sjogren’s syndrome, and sarcoidosis. [1][5]In autoimmune diseases, the immune system, which normally protects the body from foreign organisms, mistakenly attacks the body’s own tissue, causing inflammation and, in some cases, damage to myelin within the spinal cord. [1]

Multiple sclerosis (MS). An acute, rapidly progressing form of transverse myelitis sometimes signals the first attack of multiple sclerosis (MS). It is important to note, however, that studies indicate that most people who develop transverse myelitis do not go on to develop MS. [1] Transverse myelitis occurring as a sign or symptom of multiple sclerosis usually manifests on one side of the body only. [5]

Vaccinations. Rarely, transverse myelitis may develop following certain vaccinations (chickenpox and rabies). [1][5] Although it's unclear how transverse myelitis and vaccinations are related, an immune response is suggested. [4][5]

Cancers. Myelitis related to cancer (called a paraneoplastic syndrome) is uncommon. [4] However, some cancers may trigger an abnormal immune response that may lead to transverse myelitis. [1]
 
Insufficient blood flow through the blood vessels located in the spinal cord. Some cases of transverse myelitis result from spinal arteriovenous malformations (abnormalities that alter normal patterns of blood flow) or vascular diseases such as atherosclerosis that cause ischemia, a reduction in normal levels of oxygen in spinal cord tissues. When a specific region of the spinal cord becomes starved of oxygen, or ischemic, nerve cells and fibers may begin to deteriorate relatively quickly. This damage may cause widespread inflammation, sometimes leading to transverse myelitis. Most people who develop the condition as a result of vascular disease are past the age of 50, have cardiac disease, or have recently undergone a chest or abdominal operation. [1]

Last updated: 11/21/2008

How is transverse myelitis diagnosed?

Physicians diagnose transverse myelitis by taking a medical history and performing a thorough neurological examination. [1] A test known as magnetic resonance imaging (MRI) may be performed to idenify any  inflammation around the spinal cord. Physicians also may perform myelography, which involves injecting a dye into the sac that surrounds the spinal cord. The patient is then tilted up and down to let the dye flow around and outline the spinal cord while X-rays are taken. [1][4][6]

Blood tests may also be performed to rule out various disorders such as systemic lupus erythematosus, HIV infection, and vitamin B12 deficiency. In some patients with transverse myelitis, the cerebrospinal fluid that bathes the spinal cord and brain contains more protein than usual and an increased number of leukocytes (white blood cells), indicating possible infection. A spinal tap (lumbar puncture) may be performed to obtain fluid to study these factors. [1]

Last updated: 8/15/2008

How might transverse myelitis be treated?

As with many disorders of the spinal cord, no effective cure currently exists for people with transverse myelitis. Treatments are designed to manage and alleviate symptoms and largely depend upon the severity of neurological involvement. Therapy generally begins when the patient first experiences symptoms. Physicians often prescribe corticosteroid therapy during the first few weeks of illness to decrease inflammation. Although no clinical trials have investigated whether corticosteroids alter the course of transverse myelitis, these drugs often are prescribed to reduce immune system activity because of the suspected autoimmune mechanisms involved in the disorder. Corticosteroid medications that might be prescribed may include methylprednisone or dexamethasone. [1]

Chronic pain is a common complication of transverse myelitis. Nonsteroidal anti-inflammatory drugs — such as aspirin, ibuprofen (Advil, Motrin, others) and naproxen (Aleve, Naprosyn, others) — can help reduce inflammation and relieve pain. [7] Bedrest is often recommended during the initial days and weeks after onset of the disorder. [1]

Based on patient response and test results, additional therapeutic options may be added. Plasma exchange therapy is often used for those with moderate to severe transverse myelitis who don’t show much improvement after being treated with intravenous steroids. [3][7] This therapy involves removing the straw-colored fluid in which blood cells are suspended (plasma) and replacing the plasma loss with special fluids. It's not certain how this therapy helps people with transverse myelitis, but it may be that plasma exchange removes antibodies that are involved in inflammation. [7]

Some patients respond very well to intravenous cyclophosphamide (a drug often used for lymphomas or leukemia). It is very important that an experienced oncology team be involved in the administration of this drug, and patients should be monitored carefully. Ongoing treatment with chemical agents that modify immune response (such as azathioprine, methotrexate, mycophenolate or oral cyclophosphamide) can be considered for the small subset of patients that experience recurrent transverse myelitis.
[3]

Individuals with permanent physical disabilities may benefit from physical therapy, occupational therapy and vocational therapy. [1]
Last updated: 8/15/2008

What is the prognosis for individuals with transverse myelitis?

Recovery from transverse myelitis usually begins within 2 to 12 weeks of the onset of symptoms and may continue for up to 2 years. However, if there is no improvement within the first 3 to 6 months, significant recovery is unlikely. About one-third of people affected with transverse myelitis experience good or full recovery from their symptoms; they regain the ability to walk normally and experience minimal urinary or bowel effects and paresthesias. Another one-third show only fair recovery and are left with significant deficits such as spastic gait, sensory dysfunction, and prominent urinary urgency or incontinence. The remaining one-third show no recovery at all, remaining wheelchair-bound or bedridden with marked dependence on others for basic functions of daily living. Unfortunately, making predictions about individual cases is difficult. However, research has shown that a rapid onset of symptoms generally results in poorer recovery outcomes. [1] 

The majority of people with this disorder experience only one episode although in rare cases recurrent or relapsing transverse myelitis does occur. Some patients recover completely, then experience a relapse. Others begin to recover, then suffer worsening of symptoms before recovery continues. In all cases of relapse, physicians will likely investigate possible underlying causes such as MS or systemic lupus erythematosus since most people who experience relapse have an underlying disorder. [1]

Last updated: 8/15/2008

Who can I contact to learn more about transverse myelitis?

You can contact the National Institute of Neurological Disorders and Stroke (NINDS), part of the National Institutes of Health (NIH), to learn more about transverse myelitis.

NINDS provides an information page on transverse myelitis at the following web site.
http://www.ninds.nih.gov/disorders/transversemyelitis/detail_transversemyelitis.htm

NIH Neurological Institute
P.O. Box 5801
Bethesda, MD 20824
Toll-free:  800-352-9424
Phone: 301-496-5751
TTY: 301-468-5981
Email form: http://www.ninds.nih.gov/contact_us.htm
Web site:   http://www.ninds.nih.gov/

You can also contact the following organizations to obtain information about transverse myelitis.

The Transverse Myelitis Center at Johns Hopkins University
Attn: Mary Brown
Pathology 627
600 North Wolfe Street
Baltimore, MD 21287
Phone: 410.614.1522
Fax: 410.502.6736
Web site: http://hopkinsneuro.org/tm/what_we_do.cfm

Tranverse Myelitis Association
1787 Sutter Parkway
Powell, OH   43065-8806
Phone: 614-766-1806
Email: info@myelitis.org
Web site: http://www.myelitis.org

Last updated: 8/15/2008

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