Transverse myelitis (patient information)

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 * Associate Editor(s)-In-Chief: Varun Kumar, M.B.B.S.

Overview
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 simply 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.

The segment of the spinal cord at which the damage occurs determines which parts of the body are affected.

What are the symptoms of Transverse myelitis?
Transverse myelitis may be either acute (developing over hours to several days) or subacute (usually developing over 1 to 4 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 may progress 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.

Initially, people with transverse myelitis may notice that they are stumbling or dragging one foot or that their legs seem heavier than normal. Coordination of hand and arm movements, as well as arm and hand strength may also be compromised. Progression of the disease leads to full paralysis of the legs, requiring the patient to use a wheelchair.

Pain is the primary presenting symptom of transverse myelitis in approximately one-third to one-half of all patients. The pain may be localized in the lower back or may consist of sharp, shooting sensations that radiate down the legs or arms or around the torso.

Patients who experience sensory disturbances often use terms such as numbness, tingling, coldness, or burning to describe their symptoms. Up to 80 percent of those with transverse myelitis report areas of heightened sensitivity to touch, such that clothing or a light touch with a finger causes significant discomfort or pain (a condition called allodynia). Many also experience heightened sensitivity to changes in temperature or to extreme heat or cold.

Bladder and bowel problems may involve increased frequency of the urge to urinate or have bowel movements, incontinence, difficulty voiding, the sensation of incomplete evacuation, and constipation. Over the course of the disease, the majority of people with transverse myelitis will experience one or several of these symptoms.

What causes Transverse myelitis?
Researchers are uncertain of the exact causes of transverse myelitis. The inflammation that causes such extensive damage to nerve fibers of the spinal cord may result from viral infections or abnormal immune reactions. Transverse myelitis also may occur as a complication of syphilis, measles, Lyme disease, and some vaccinations, including those for chickenpox and rabies. Cases in which a cause cannot be identified are called idiopathic.

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, and rubella. Bacterial skin infections, middle-ear infections (otitis media), and Mycoplasma pneumoniae (bacterial pneumonia) have also been associated with the condition.

In 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. 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. 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.

Because some affected individuals also have autoimmune diseases such as systemic lupus erythematosus, Sjogren’s syndrome, and sarcoidosis, some scientists suggest that transverse myelitis may also be an autoimmune disorder. In addition, some cancers may trigger an abnormal immune response that may lead to transverse myelitis.

Who is at highest risk?
Transverse myelitis occurs in adults and children, in both genders, and in all races. No familial predisposition is apparent. A peak in incidence rates (the number of new cases per year) appears to occur between 10 and 19 years and 30 and 39 years. Although only a few studies have examined incidence rates, it is estimated that about 1,400 new cases of transverse myelitis are diagnosed each year in the United States, and approximately 33,000 Americans have some type of disability resulting from the disorder.

Diagnosis
Physicians diagnose transverse myelitis by taking a medical history and performing a thorough neurological examination. Because it is often difficult to distinguish between a patient with an idiopathic form of transverse myelitis and one who has an underlying condition, physicians must first eliminate potentially treatable causes of the condition. when a spinal cord problem is suspected, physicians seek first to rule out structural lesions (damaged or abnormally functioning areas) that could cause spinal cord compression or otherwise affects its function. Such potential lesions include tumors, herniated or slipped discs, stenosis (narrowing of the canal that holds the spinal cord), abscesses, and abnormal collections of blood vessels. To rule out such lesions and check for inflammation of the spinal cord, patients often undergo magnetic resonance imaging (MRI), a procedure that provides a picture of the brain and spinal cord. The spinal cord MRI will almost always confirm the presence of a lesion within the spinal cord, whereas the brain MRI may provide clues to other underlying causes, especially MS. If an MRI is not possible (for example, if the patient has a pacemaker), physicians may consider other diagnostic tests such as computed tomography (CT) of the spine with or without 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.

Blood tests may be performed to rule out various disorders such as systemic lupus erythematosus, HIV infection, vitamin B12 deficiency, and many others  A blood test for NMO, called NMO-IgG, is also necessary. 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). A spinal tap may be performed to obtain fluid to study these factors, exclude infections, and to look for markers of diseases such as MS.

If none of these tests suggests a specific cause, the patient is presumed to have idiopathic transverse myelitis.

Treatment options
As with many disorders of the spinal cord, no effective cure currently exists for people with transverse myelitis. Treatments are designed to reduce spinal cord inflammation and manage and alleviate symptoms.. Physicians often prescribe anti-inflammatory corticosteroid therapy soon after the diagnosis is made in order to decrease inflammation and hopefully improve the chances and speed of neurological recovery. 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 intravenous methylprednisone or dexamethasone (usually for about 5 days; in some cases, oral prednisone is used for a period of time afterwards). In severe cases that do not appear to respond to corticosteroid treatment, other therapies such as plasma exchange or drug therapies may be used to try to salvage neurological function. General painkillers may be prescribed for any pain the patient may have. And bed rest is often recommended during the initial days and weeks after onset of the disorder.

Following initial therapy, the most critical part of the treatment for this disorder consists of keeping the patient’s body functioning while hoping for either complete or partial spontaneous recovery of the nervous system. This may require placing the patient on a respirator in the uncommon scenario where breathing is significantly affected. Patients are most often treated in a hospital or in a rehabilitation facility where a specialized medical team can prevent or treat problems that afflict paralyzed patients. Often, even before recovery begins, caregivers may be instructed to move patients’ limbs manually to help keep the muscles flexible and strong, and to reduce the likelihood of pressure sores developing in immobilized areas. Later, if patients begin to recover limb control, physical therapy begins to help improve muscle strength, coordination, and range of motion.

Rehabilitation
Today, most rehabilitation programs attempt to address the emotional dimensions along with the physical problems resulting from permanent disability. Patients typically consult with a range of rehabilitation specialists, who may include physiatrists (physicians specializing in physical medicine and rehabilitation), physical therapists, occupational therapists, vocational therapists, and mental health care professionals.

Physical Therapy: Physiatrists and physical therapists treat disabilities that result from motor and sensory impairments. Their aim is to help patients increase their strength and endurance, improve coordination, reduce spasticity and muscle wasting in paralyzed limbs, and regain greater control over bladder and bowel function through various exercises. Physiatrists and physical therapists teach paralyzed patients techniques for using assistive devices such as wheelchairs, canes, or braces as effectively as possible. Paralyzed patients also learn ways to avoid developing painful pressure sores on immobilized parts of the body, which may lead to increased pain or systemic infection. In addition, physiatrists and physical therapists are involved in pain management. A wide variety of drugs now exist that can alleviate the pain that results from spinal cord injuries such as those caused by transverse myelitis. These include nonsteroidal anti-inflammatory drugs such as ibuprofen or naproxen; antidepressant drugs such as amitryptyline (tricyclic) and sertraline (a selective serotonin reuptake inhibitor); muscle relaxants such as baclofen or tizanidine, and anticonvulsant drugs such as gabapentin, pregabalin, and carbamazepine.

Occupational Therapy: Occupational therapists help individuals learn new ways to maintain or rebuild their independence by participating in meaningful, self-directed, goal-oriented, everyday tasks (occupations) such as bathing and dressing. They teach people how to function at the lightest level possible, by developing compensatory strategies, suggesting changes in their homes to improve safety (such as installing grab bars in bathrooms), changing obstacles in their environment that interfere with normal activity, and instructing on how to use assistive devices.

Vocational Therapy: In addition to acquainting people with their rights as defined under the Americans with Disabilities Act of 1990 and helping people develop and promote work skills, vocational therapists identify potential employers, assist in job searches, and act as mediators between employees and employers to secure reasonable workplace accommodations.

Where to find medical care for Transverse myelitis?
Directions to Hospitals Treating Transverse myelitis

What to expect (Outlook/Prognosis)?
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.

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 evaluate possible underlying causes such as MS. NMO, or systemic lupus erythematosus since most people who experience relapse have an identifiable underlying disorder. People with a recurrent relapsing disorder will usually require some type of ongoing therapy that modulates or supresses the immune system. The propose of such therapies is to reduce the chance of future relapses.