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Transverse Myelitis


National Organization for Rare Disorders, Inc.

Disorder Subdivisions

  • Ascending Myelitis
  • Brown-Sequard Syndrome
  • Concussion Myelitis
  • Foix-Alajouanine Myelitis
  • Subacute Necrotizing Myelitis
  • Funicular Myelitis
  • Systemic Myelitis

Related Disorders List

Information on the following diseases can be found in the Related Disorders section of this report:

  • Spinal Stenosis
  • Cervical Spondylosis

General Discussion

Transverse myelitis is a neurological disorder of the spine caused by inflammation across the spinal cord. It is sometimes associated with the term myelopathy, which refers to any disorder of the spinal cord. However, transverse myelitis is a more specific term for inflammation (myelitis) across the width of the spinal cord (transverse) that results in changed function below this level while function remains normal above. Symptoms are related to movement and sensory functions. This disorder occurs in both adults and children, and typically begins with a rather rapid development of symptoms over the course of several hours, days, or weeks. Symptoms may include lower back pain, weakness in the legs and arms, sensory disturbance, spasms leading to gradual paralysis, and bowel or bladder disfunction.

In most cases, this is a disorder that occurs on a single occasion, although a small number of individuals may experience recurrence. The initial occurrence may be followed, over a period of several weeks or months, by a period of recovery, although this does not happen in all cases. There is considerable variability in the degree of recovery achieved. Transverse myelitis is sometimes associated with other diseases, including systemic autoimmune diseases like systemic lupus erythematosis and sarcoidosis. It may be occur following viral or bacterial infections, especially those associated with a rash, spinal cord injuries, or immune reactions.

Symptoms

Symptoms are related to the specific area within the spinal cord where the inflammation occurs. In general, they may include pain (often in the lower back), weakness of the legs (and, sometimes, arms), loss of sensation below the affected area, and bowel or bladder dysfunction. Symptoms may also include headache, loss of appetite, and numbness or tingling of legs.

Eventually, loss of sensation may be severe, associated with sensorimotor paralysis below the level of the lesion, urinary retention, and loss of sexual function and/or bowel (sphincter) control. Affected individuals may experience thick, rough, dry skin below the spinal lesion, and tendon reflexes may be lost.

The affected individual may notice that his or her legs seem heavier than usual, or that he or she stumbles or drags one foot. Coordination of hand and arm movements may be affected. Over time, progression of the disease may lead to full paralysis of the legs, requiring the patient to use a wheelchair.

Following the initial development of the disease, there may be a period of recovery, although this does not occur in all cases. The improvement, which may occur within the first one to three months, can range from slight to good. Individuals who do not show signs of improvement within the first three months may be more likely to experience long-term deficients from the disease.

Causes

Although the exact causes of transverse myelitis are not fully understood, the inflammation that causes damage to nerve fibers of the spinal cord can be caused by viral infections, spinal cord injuries, immune reactions, or insufficient blood flow through the blood vessels in the spinal cord. It can occur as a complication of loss of nerve sheath tissue (demyelination), particularly in optic neuromyelitis or multiple sclerosis. It can also be a complication of reactions to some vaccinations, including those for smallpox, measles, or chickenpox.

Transverse myelitis can be associated with other diseases including acute disseminated encephalomyelitis (ADEM), which is a serious inflammation of the brain and spinal cord. It may be associated with many different diseases, including chickenpox, measles, rabies, smallpox, Lyme disease, Sjogren syndrome, syphilis, and multiple sclerosis.

Affected Populations

Transverse myelitis can begin at any time of life. It affects both children and adults. It seems to occur equally among females and males, and among persons of all ethnicities. There does not appear to be a genetic component to the disorder since no familial predisposition has been noted.

There appear to be two age groups in which the largest number of new cases occurs. These peaks are between ages 10 and 19 and between ages 30 and 39. The National Institute of Neurological Disorders and Stroke (NINDS) suggests that there are about 1,400 new cases of transverse myelitis in the USA per year and that 33,000 persons in the USA have some form of disability as a result of transverse myelitis.

Related Disorders

Symptoms of the following disorders can be similar to those of transverse myelitis. Comparisons may be useful for a differential diagnosis:

Guillain-Barre syndrome is a very rare, rapidly progressive disorder that consists of inflammation of the nerves (polyneuritis) and, usually, muscle weakness, often progressing to paralysis. Although its precise cause is unknown, a viral or respiratory infection precedes the onset of the syndrome in about half of the cases. This has led to the theory that Guillain Barre syndrome may be an autoimmune disease (a disease that occurs when an abnormal immune system response is triggered). Damage to the covering of nerve cells (myelin) and nerve axons (the extension of the nerve cell that conducts impulses away from the nerve cell body) results in delayed nerve signal transmission. There is a corresponding weakness in the muscles that are supplied or innervated by the damaged nerves.

Standard Therapies

There is currently no effective cure for people with transverse myelitis. Treatment is symptomatic and supportive. During the first few weeks of the illness, corticosteroid drugs may help in treating spinal cord inflammation. Corticosteroid medications that might be prescribed include methylprednisone and dexamethasone. Pain medications and bedrest may also be helpful during the early weeks of the illness. People with disability that continues after the period of initial illness may benefit from physical therapy to improve muscle strength, coordination, and range of motion.

Investigational Therapies

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
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

The National Institute of Neurological Disorders and Stroke (NINDS) of the National Institutes of Health (NIH) conducts research at the NIH Clinical Center in Bethesda and supports research at other locations on spinal cord disorders and demyelinating diseases (ones in which the lining of nerve tissues is damaged) such as transverse myelitis. NINDS researchers are seeking to clarify the role of the immune system in the initiation of autoimmune diseases, to develop strategies to repair spinal cords in which the myelin lining has been damaged, and to develop electrical and mechanical devices to help individuals with spinal cord damage compensate for lost function.

References

TEXTBOOKS
Beers MH, Berkow R, eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:1483.

Berkow R, ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:326.

Rowland LP. Ed. Merritt's Neurology. 10th ed. Lippincott Williams & Wilkins. Philadelphia, PA. 2000:152.

Adams RD, Victor M, Ropper AA. Eds. Principles of Neurology. 6th ed. McGraw-Hill Companies. New York, NY; 1997:1242.

REVIEW ARTICLES
Chen HC, Lai JH, Juan CJ, et al. Longitudinal myelitis as an initial manifestation of systemic lupus erythematosus. Am J Med Sci. 2004;327:105-08.

Andronikou S, Albuquerque-Jonathan G, Wilmhurst J, et al. MRI findings in acute idiopathic transverse myelopathy in children. Pediatr Radiol. 2003;33:624-29.

Boos J, Davis LE. Smallpox and smallpox vaccination: neurological implications. Neurology. 2003;60:1241-45.

Cree BA, Goodin DS, Hauser SL. Neuromyelitis optica. Semin Neurol. 2002;22:105-22.

Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology. 2002;59:499-505.

Sherer Y, Hassin S, Shoenfeld Y, et al. Transverse myelitis in patients with antiphospholipid antibodies—the importance of early diagnosis and treatment. Clin Rheumatol. 2002;21:207-10.

JOURNAL ARTICLES
Ghezzi A, Bergamaschi R, Martinelli V, et al. Clinical characteristics, course and prognosis of relapsing Devic's Neuromyelitis Optica. J Neurol. 2004;251:47-52.

Krishnan C, Kaplin AI, Deshpande DM, et al. Transverse Myelitis: pathogenesis, diagnosis and treatment. Front Biosci. 2004;9:1483-99.

D'Cruz DP, Mellor-Pita S, Joven B, et al. Transverse myelitis as the first manifestation of systemic lupus erythematosus. Am J Med Sci. 2004;31:280-85.

Torabi AM, Patel RK, Wolfe GI, et al. Transverse myelitis in systemic sclerosis. Arch Neurol. 2004;61:126-28.

Harzheim M, Schlegel U, Urbach H, et al. Descriminatory features of acute transverse myelitis: a retrospective analysis of 45 patients. J Neurol Sci. 2004;217:217-23.

Miyazawa R, Ikeuchi Y, Tomomasa T, et al. Determinants of prognosis of acute transverse myelitis in children. Pediatr Int. 2003;45:512-16.

Kim KK. Idiopathic recurrent transverse myelitis. Arch Neurol. 2003;60:1290-94.

Defresne P, Hollenberg H, Husson B, et al. Acute transverse myelitis in children: clinical course and prognostic factors. J Child Neurol. 2003;18:401-06.

Oleson CV, Sivalingham JJ, O'Neill BJ, et al. Transverse myelitis secondary to coexistent Lyme disease and babesiosis. J Spinal Cord Med. 2003;26:168-71.

Wingerchuk DM, Weinshenker BG. Neuromyelitis optica: clinical predictors of a relapsing course and survival. Neurology. 2003;60:848-53.

Schimmel MS, Schlesinger Y, Berger I, et al. Transverse myelitis: unusual sequelae of neonatal group B streptococcus disease. J Perinatol. 2002;13:375-80.

FROM THE INTERNET
Transverse Myelitis Fact Sheet. National Institute of Neurological Disorders and Stroke (NINDS). Reviewed September 16, 2002.
www.ninds.nih.gov/health_and_medical/pubs/transverse_myelitis.htm

Transverse Myelitis Center, Department of Neurology, Johns Hopkins Medicine. ©2003.
The Johns Hopkins Myelitis Center. www.hopkinsmedicine.org/jhtmc
What is Transverse Myelitis? www.hopkinsmedicine.org/jhtmc/definitions.htm
Clinical Aspects of Transverse Myelitis. www.hopkinsmedicine.org/jhtmc/clinical.htm
Treatment Options for TM. www.hopkinsmedicine.org/jhtmc/treatment.htm
Diseases Associated with TM. www.hopkinsmedicine.org/jhtmcdiseases/index.htm
Information for Physicians. www.hopkinsmedicine.org/jhtmc/for_MDs/pathology.htm
TM References. www.hopkinsmedicine.org/jhtmc/references.htm

Acute Transverse Myelitis. The Merck Manual/Second Home Edition. ©2004. 2pp.
www.merck.com/mrkshared/mmanual_home2/sec06/ch093/ch093h.jsp

Resources

Transverse Myelitis Association
1787 Sutter Parkway
Powell, OH 43065-8806
USA
Tel: (614)766-1806
Email: ssiegel@myelitis.org
Internet: http://www.myelitis.org

Spinal Cord Society
19051 County Hwy. 1
Fergus Falls, MN 56537
USA
Tel: (218)739-5252
Fax: (218)739-5262
Email: scs-nc@nc.rr.com
Internet: http://www.spinalcordsociety.com

American Paraplegia Society
75-20 Astoria Boulevard
Jackson Heights, NY 11370-1177
USA
Tel: (718)803-3782
Fax: (718)803-0414
Email: aps@UnitedSpinal.ORG
Internet: http://www.apssci.org

National Spinal Cord Injury Association
6701 Democracy
Suite 300-9
Bethesda, MD 20817
USA
Tel: (301)214-4006
Fax: (301)881-9817
Tel: (800)962-9629
Email: info@spinalcord.org
Internet: http://www.spinalcord.org

National Institute of Neurological Disorders and Stroke (NINDS)
31 Center Drive
8A07
Bethesda, MD 20892-2540
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
Email: braininfo@ninds.nih.gov
Internet: http://www.ninds.nih.gov/

MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
150 Custer Court
Green Bay, WI 54301-1243
USA
Tel: (920)336-5333
Fax: (920)339-0995
Tel: (877)336-5333
Email: mums@netnet.net
Internet: http://www.netnet.net/mums/

Christopher Reeve Paralysis Foundation
500 Morris Ave
Springfield, NJ 07081
USA
Tel: (973)379-2690
Fax: (973)912-9433
Tel: (800)225-0292
Email: info@crof.org
Internet: http://www.crpf.org, http://www.paralysis.org

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)519-3194
Fax: (240)632-9164
Tel: (888)205-2311
TDD: (888)205-3223
Email: gardinfo@nih.gov
Internet: http://www.genome.gov/10000409

Devic's Support
107 Woodside Manor
Carmichaels, PA 15320
Email: gmitchell@myelitis.org or grace53a@gmail.com
Internet: http://groups.google.com/group/Devics-support?pli=1

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). CIGNA members can access the complete report by logging into myCIGNA.com. For non-CIGNA members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  4/22/2008
Copyright  1988, 1989, 1996, 2004 National Organization for Rare Disorders, Inc.



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