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Transverse myelitis

 
Medical Encyclopedia: Transverse Myelitis

Definition

Transverse myelitis (TM) is an uncommon neurological syndrome caused by inflammation (a protective response which includes swelling, pain, heat, and redness) of the spinal cord, characterized by weakness, back pain, and bowel and bladder problems. It affects one to five persons per million.

Description

TM affects the entire thickness of the spinal cord, producing both sensory and movement problems. It is believed to be linked to the immune system, which may be prompted to attack the body's own spinal cord. Striking rapidly without warning, its effects can be devastating.

— Carol A. Turkington



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Neurological Disorder:

Transverse myelitis

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Definition

Transverse myelitis is an inflammation of the full width of the spinal cord that disrupts communication to the muscles, resulting in pain, weakness, and muscle paralysis.

Description

The symptoms of transverse myelitis are due to damage and/or destruction of the myelin sheath, the fatty white covering of nerve fibers that serves both to insulate the nerve fibers and to speed nervous conduction along them. Areas of missing myelin and areas of scarring along the affected nerves result in slowed or disrupted nervous conduction and muscle dysfunction.

Transverse myelitis may have a gradual onset or a remarkably quick onset. Symptoms of transverse myelitis may reach their peak within 24 hours of onset for some patients (considered the hyperacute form of the condition). Other patients experience a more gradual increase in symptom severity, with peak deficits occurring days (acute form of transverse myelitis) to weeks (subacute form of transverse myelitis) after the initial symptoms first presented. Patients with the quicker onset form and who experience more severe initial symptoms tend to have more complications and a greater likelihood of permanent disability.

Transverse myelitis often occurs in people who are recovering from a recent viral illness, including chickenpox, herpes simplex, cytomegalovirus, Epstein-Barr, influenza, and measles. When this association is present, the condition often follows the more sudden hyperacute course.

Demographics

In the United States, there are only about 4.6 cases of transverse myelitis per million people per year. In the Unites States, about 1,400 people a year develop transverse myelitis; about 33,000 people in the United States have disabilities due to transverse myelitis. Individuals of all ages can be affected; reports have been made of patients ranging from the age of six months to 88 years. The peak ages appear to be 10-19 years and 30-39 years.

About 30-60% of all cases of transverse myelitis occur in individuals who have just recovered (within the previous 8 weeks) from a relatively minor viral infection. Recent vaccination is another risk factor for transverse myelitis. Other individuals at higher risk for transverse myelitis include patients with preexisting autoimmune diseases (such as multiple sclerosis, systemic lupus erythematosus, or Devic's disease); patients with recent histories of infections such as Lyme disease, tuberculosis, or syphilis; and intravenous drug abusers who inject heroine and/or amphetamines.

Causes and symptoms

Although the specific mechanism of transverse myelitis has not been delineated, the basic cause is thought to be an autoimmune response. Under normal conditions, the immune system reacts to the presence of a viral or bacterial illness by producing a variety of immune cells designed to attack the invading viruses or bacteria. Unfortunately, in the case of transverse myelitis, the immune cells mistake the body's own tissues as foreign, and attack those tissues as well. These errant immune cells are called autoantibodies; that is, antibodies that actually attack the body's own tissues.

Symptoms of transverse myelitis can develop over several hours, days, or weeks. The types of symptoms and their severity are dependent on the area of the spinal cord affected. When the transverse myelitis occurs in the neck, the arms and legs will be affected; when the transverse myelitis occurs lower in the back, only the legs will be affected.

Symptoms of transverse myelitis often begin with back pain, headache, achy muscles, flu-like symptoms, and stiff neck. Over hours or days, symptoms expand to include loss of sensation, numbness, dysesthesia (sensations of burning, lightning flashes of pain, prickly pinpoints), muscle weakness, partial or complete paralysis, and impaired bladder and bowel function. Symptoms of weakness and then paralysis usually begin in the feet, ascending over time to the legs, and then to the trunk and arms when the lesion is in the neck. Symptoms are bilateral, meaning that they affect both sides of the body simultaneously. Over time, muscles become increasingly tight and spastic, further limiting mobility. When the muscles of respiration are affected, breathing can be compromised.

Diagnosis

Diagnosis involves meeting specific symptom criteria, as well as demonstrating spinal cord involvement with MRI scanning and examination of cerebrospinal fluid. Symptom criteria include the evolution of symptoms peaking over four hours to 21 days, with symptoms clearly traceable to spinal cord dysfunction, and including muscle weakness or paralysis and sensory defects such as numbness occurring on both sides of the body. The presence of a spinal cord tumor or another condition that is exerting pressure on the spinal cord, vitamin B12 deficiency, or a history of radiation therapy to or cyclophosphamide injection into the spinal cord excludes the possibility of a diagnosis of transverse myelitis.

Treatment team

The mainstay of the treatment team for patients with transverse myelitis will be a neurologist. A rheumatologist, specializing in autoimmune illness, may also be consulted. In order to regain maximum function, a physiatrist (a physician specializing in rehabilitation medicine) may be required, as well as the services of both physical and occupational therapists.

Treatment

Treatment is aimed at calming the immune response that caused the spinal cord injury in the first place. To this end, high doses of intravenous and then oral steroids are the first-line treatments for transverse myelitis. In severe cases of transverse myelitis, the very potent immunosupressant cyclophosphamide may be administered. In patients with moderately severe transverse myelitis unimproved by five to seven days of steroid treatment, a procedure called plasma exchange may be utilized. This procedure involves removing blood from the patient, and separating it into the blood cells and the plasma (fluid). The blood cells are then mixed into a synthetic plasma replacement solution and returned to the patient. Because the immune cells are in the plasma, this effectively removes the damaging immune cells from the body, hopefully quelling the myelin destruction.

Treatments to reverse the process involved in transverse myelitis should be attempted for about six months from the onset of the condition. After that point, treatment efforts should be shifted to effective rehabilitation.

Pain and other dysesthesias (uncomfortable sensations, such as burning, pins-and-needles, or electric shock sensations) are treated with a variety of medications, such as gabapentin, carbamazepine, nortriptyline, or tramadol. Another treatment for pain and dysesthesias is transcutaneous electrical nerve stimulation, called TENS therapy. This involves the use of a device that stimulates the painful area with a small electrical pulse, which seems to disrupt the painful sensation.

Because constipation and urinary retention are frequent problems in the patient with transverse myelitis, medications may be necessary to treat these problems. Oxybutinin, hyoscyamine, tolterodine, and propantheline can treat some of the bladder problems common to transverse myelitis patients. When urinary retention is an issue, sacral nerve stimulation may help the patient avoid repeated bladder catheterizations. Dulcolax, senekot, and bisacodyl can help improve constipation.

Tight, spastic muscles may improve with baclofen, tizanidine, or diazepam. When these medications are given orally, they sometimes result in untenable side effects.

Recovery and rehabilitation

Rehabilitation has both short-and long-term components. Even in the earliest stages of the condition, passive exercises should be performed. Passive exercises involve a physical therapist putting a particular muscle group or joint through range of motion and strengthening exercise, even when the patient cannot assist in its movement. During the recovery phase, the patient should be given progressive exercises to improve strength and range of motion, and to attempt to regain mobility. Physical therapists can also be helpful with pain management, using such techniques as heat and/or cold application, nerve stimulation, ultrasound, and massage. Physical therapy may also be helpful to retrain muscles necessary for improved bladder and bowel control and relief of constipation and urinary retention. Occupational therapists can help the patient relearn old skills for accomplishing the activities of daily living, or strategize new techniques that take into account the patient's disabilities.

Braces or assistive devices such as walkers, wheelchairs, crutches, or canes may be necessary during rehabilitation or permanently.

Prognosis

The area on the spinal cord affected by transverse myelitis will determine the individual's level of functioning. The higher-up the lesion, the greater the disability. High cervical lesions will require complete care; as lesions drop lower and lower in the cervical, thoracic, or lumbar region, the chance to participate in self-care or even to ambulate increases.

Recovery from transverse myelitis seems to follow the law of thirds: about a third of all patients make a full recovery from their level of functioning at the condition's peak, a third make a partial recovery, and a third make no recovery at all. Most patients make a good or even a complete recovery within one to three months of the onset of their symptoms. Patients who have not begun to improve by month three after symptom onset usually will not accomplish a complete recovery from their disability. Factors that do not bode well include abrupt onset of symptoms, prominent pain upon onset, and severe disability and deficit at the peak of the condition.

Resources

BOOKS

Aminoff, Michael J. "Inflammatory disorders affecting the spinal cord." In Cecil Textbook of Internal Medicine, edited by Lee Goldman, et al. Philadelphia: W. B. Saunders Company, 2000.

Schneider, Deborah Ross. "Transverse Myelitis." In Essentials of Physical Medicine and Rehabilitation, 1st ed., edited by Walter R. Frontera. Philadelphia: Hanley and Belfus, 2002.

PERIODICALS

Transverse Myelitis Consortium Working Group. "Proposed diagnostic criteria and nosology of acute transverse myelitis." In Neurology 59, no. 4 (27 August 2002): 499–505

WEBSITES

National Institute of Neurological Disorders and Stroke (NINDS). NINDS Transverse Myelitis Information Page. July 1, 2001 (June 10, 2004). http://www.ninds.nih.gov/health_and_medical/disorders/transversemyelitis_doc.htm.

ORGANIZATIONS

Transverse Myelitis Association. 1787 Sutter Parkway, Powell, OH 43065. (614) 766-1806. ssiegel@myelitis.org. http://www.myelitis.org/index.html.

The Johns Hopkins Transverse Myelitis Center. 600 N. Wolfe Street, Baltimore, MD 21287. (410) 502-7099; Fax: (410) 502-6736. dkerr@jhmi.edu. http://www.hopkinsmedicine.org/jhtmc/.

Rosalyn Carson-DeWitt, MD


Oncology Encyclopedia: Transverse Myelitis
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Key Terms: Autoimmune disease, Catheter, Infectious disease, Spinal cord, Spinal cord compression.

Description

Transverse myelitis (TM) is an inflammation or infection of the spinal cord in which the effect of the lesion spans the width of the entire spinal cord at a given level. The spinal cord consists of four regions: the cervical (neck), followed by the thoracic (chest), the lumbar (lower back) and the sacral (lowest back). TM can occur in any of these regions. The disease is uncommon, but not rare, as it occurs in one to five persons per million population in any given year in the United States. It is equally diagnosed in both adults and children. TM may occur by itself or in conjunction with other illnesses such as viral or bacterial infectious diseases, autoimmune diseases such as multiple sclerosis, vascular illnesses such as thrombosis, and cancer.

The symptoms of TM depend on the level of spinal cord lesion with sensation usually diminished below the spinal cord level affected. Some patients experience tingling sensations or numbness in the legs with bladder control also being disturbed. The condition is usually diagnosed following magnetic resonance imaging (MRI) or computed tomography (CT) with "spinal taps" (lumbar punctures) taken for additional analysis. Recovery depends on the general health status of the patient and is usually considered unlikely if no improvement is observed within three months.

Causes

The exact cause of TM is unknown but research results point to autoimmune deficiencies, meaning that the patient's own immune system abnormally attacks the spinal cord, resulting in inflammation and tissue damage.

There is also evidence suggesting that TM occurs as a result of spinal cord compression by tumors or as a result of direct spinal cord invasion by infectious agents, especially the human immunodeficiency virus (HIV) and the human T-lymphotropic virus type I (HTLV-1).

TM is also listed among the spinal cord disorders occurring in patients diagnosed with AIDS.

Treatments

There is no specific treatment for transverse myelitis. Treatment of the illness is largely symptomatic, meaning that it depends on the specific symptoms of the patient. The region in which the spinal cord has been infected is critical but a course of intravenous steroids is generally prescribed at the onset of treatment.

Treatment of the bladder function impairment resulting from TM include drugs, external catheters for men and padding for women, with surgery recommended in certain cases. A common TM side effect is difficulty with stool evacuation and this condition can be treated by diets that include stool softeners and fiber.

As a result of TM, muscle groups below the affected level may become spastic. Treatment of spasticity usually involves prescriptions of drugs such as Baclofen (Lioresal), which stops reflex activity, and Dantrolene sodium (Dantrium) which acts directly on muscle. A new very well-tolerated drug, Tizanidine, has also recently been introduced in the United States. Muscle pain is generally treated with analgesics such as acetaminophen (Tylenol) or ibuprofen (Naprosyn, Aleve, Motrin). Nerve disorders might be treated with anticonvulsant drugs such as carbamazepine, phenytoin or gabapentin (Tegretol, Dilantin, Neurontin).

Alternative and Complementary Therapies

Individuals with TM may experience serious difficulty with common tasks such as dressing, bathing and eating. Complementary TM therapies may accordingly include a course of physical therapy so as to help patients recover mobility. This can be achieved with special exercises, canes, walkers and custom-designed braces.

After the acute phase, people with TM start the rehabilitation process. During this period, the focus of care is shifted from designing an effective TM treatment to learning to cope with a serious disease. TM patients must learn to cope with the loss of abilities which healthy people take for granted and this process is necessarily harder if TM is associated with AIDS or another serious autoimmune disease. Resources that may help this required adjustment are psychological assistance from counselors, relatives and friends, and making contact with TM support groups. The Transverse Myelitis Association may also be contacted: 3548 Tahoma Pl. West, Tacoma, WA 98466-2141 (info@myelitis.org; www.myelitis.org) Phone:253-565-8156.

Resources

Books

Beers, M.H., and R. Berkow, editors. The Merck Manual of Diagnosis and Therapy. 17th ed. Whitehouse Station, NJ: Merck Research Laboratories, 1999.

Organizations

National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health. NIH Neurological Institute. P.O. Box 5801, Bethesda, MD 20824. (800) 352-9424. .

Transverse Myelitis Association. 3548 Tahoma Pl. West, Tacoma, WA 98466-2141. (253) 565-8156. .

—Monique Laberge, Ph.D.

Wikipedia: Transverse myelitis
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Transverse myelitis
Classification and external resources
ICD-10 G37.3
ICD-9 323.82
DiseasesDB 13265
MeSH D009188

Transverse myelitis is a neurological disorder caused by an inflammatory process of the grey and white matter of the spinal cord, and can cause axonal demyelination.

Contents

Presentation

This demyelination arises idiopathically following infections or vaccination, or due to multiple sclerosis. One major theory of the cause is that an immune-mediated inflammation is present as the result of exposure to a viral antigen.

The lesions are inflammatory, and involve the spinal cord on both sides. With acute transverse myelitis, the onset is sudden and progresses rapidly in hours and days. The lesions can be present anywhere in the spinal cord, though it is usually restricted to only a small portion.

Causes

In some cases, the disease is presumed to be caused by viral infections or vaccinations and has also been associated with spinal cord injuries, immune reactions, schistosomiasis and insufficient blood flow through spinal cord vessels. Acute myelitis accounts for 4 to 5 percent of all cases of neuroborreliosis.[1] Symptoms include weakness and numbness of the limbs as well as motor, sensory, and sphincter deficits. Severe backpain may occur in some patients at the onset of the disease. Treatment is usually symptomatic only, corticosteroids being used with limited success. A major differentiation or distinction to be made is a similar condition due to compression of the spinal cord in the spinal canal, due to disease of the surrounding vertebral column. An urgent MRI is thus indicated.

Another possible cause is dissection of the Aorta, extending into one or more of the spinal arteries.

Prognosis

Prognosis for complete recovery is generally poor. Recovery from transverse myelitis usually begins between weeks 2 and 12 following onset and may continue for up to 2 years in some patients, many of whom are left with considerable disabilities. Some patients show no signs of recovery ever.[2] However, if treated early, some patients experience complete or near complete recovery.

Symptoms and signs

The symptoms and signs depend upon the level of the spinal cord involved and the extent of the involvement of the various long tracts. In some cases, there is almost total paralysis and sensory loss below the level of the lesion. In other cases, such loss is only partial.

  • If area is involved, all four limbs may be involved and there is risk of respiratory paralysis (segments C3,4,5 to diaphragm).
  • Lesions of the lower cervical (C2-T1) region will cause a combination of upper and lower motor neuron signs in the upper limbs, and exclusively upper motor neuron signs in the lower limbs.
  • A lesion of the thoracic spinal cord (T1-12) will produce a spastic paraplegia.
  • A lesion of the lower part of the spinal cord (L1-S5) often produces a combination of upper and lower motor neuron signs in the lower limbs.

The degree and type of sensory loss will depend upon the extent of the involvement of the various sensory tracts, but there is often a "sensory level" (at the sensory segmental level of the spinal cord below which sensation to pin or light touch is impaired). This has proven to be a reasonably reliable sign of the level of the lesion. Bladder paralysis often occurs and urinary retention is an early manifestation. Considerable pain often occurs in the back, extending laterally to involve the sensory distribution of the diseased spinal segments—so-called "radicular pain." Thus, a lesion at the T8 level will produce pain radiating from the spine laterally along the lower costal margins. These signs and symptoms may progress to severe weakness within hours. (Because of the acuteness of this lesion, signs of spinal shock may be evident, in which the lower limbs will be flaccid and areflexic, rather than spastic and hyperreflexic as they should be in upper motor neuron paralysis.

Some patients have also described the feeling of their abdominal area being in a binder.

However, within several days, this spinal shock will disappear and signs of spasticity will become evident.

Differential diagnosis

The three main conditions to be considered in the differential diagnosis are: acute spinal cord trauma, acute compressive lesions of the spinal cord such as epidural metastatic tumour, and infarction of the spinal cord, usually due to insufficiency of the anterior spinal artery. Lyme disease serology is indicated in patients with transverse myelitis keeping in mind that dissociation in Lyme antibody titers between the blood and the CSF is possible.[3]

From the symptoms and signs, it may be very difficult to distinguish acute transverse myelitis from these conditions and it is almost invariably necessary to perform an emergency magnetic resonance imaging (MRI) scan or computerised tomographic (CT) myelogram. Before doing this, routine x-rays are taken of the entire spine, mainly to detect signs of metastatic disease of the vertebrae, that would imply direct extension into the epidural space and compression of the spinal cord. Often, such bony lesions are absent and it is only the MRI or CT that discloses the presence or absence of a compressive lesion.

A family physician seeing such a patient for the first time should immediately arrange transfer to the care of a neurologist or neurosurgeon who can urgently investigate the patient in hospital. Before arranging this transfer, the physician should be certain that respiration is not affected, particularly in high spinal cord lesions. If there is any evidence of this, methods of respiratory assistance must be on hand before and during the transfer procedure. The patient should also be catheterized to test for and, if necessary, drain an over-distended bladder. A lumbar puncture can be performed after the MRI or at the time of CT myelography. Steroids are often given in high dose at the onset, in hope that the degree of inflammation and swelling of the cord will be lessened, but whether this is truly effective is still debated.

Unfortunately, the prognosis for significant recovery from acute transverse myelitis is poor in approximately 80% of the cases; that is, significant long-term disabilities will remain. Approximately 5% of these patients will, in later months or years, show lesions in other parts of the central nervous system, indicating, in retrospect, that this was a first attack of multiple sclerosis. [4]

See also

References

  1. ^ Blanc F, Froelich S, Vuillemet F, Carré S, Baldauf E, de Martino S, Jaulhac B, Maitrot D, Tranchant C, de Seze J. Myelite aigue et neuroborreliose [Acute myelitis and Lyme disease]. Rev Neurol (Paris). 2007;163(11):1039-47.
  2. ^ Transverse Myelitis Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS)
  3. ^ Walid MS, Ajjan M, Ulm AJ. Subacute transverse myelitis with Lyme profile dissociation. GMS Ger Med Sci. 2008;6:Doc04.[1]
  4. ^ Transverse myelitis. Retrospective analysis of 33 cases, with differentiation of cases associated with multiple sclerosis and parainfectious events

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