Subacute Sclerosing Panencephalitis

Subacute Sclerosing Panencephalitis

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Subacute Sclerosing Panencephalitis 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.

Synonyms

  • Panencephalitis, Subacute Sclerosing
  • SSPE
  • Dawson's Disease
  • Dawson's Encephalitis

Disorder Subdivisions

  • None

General Discussion

Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder characterized by inflammation of the brain (encephalitis). The disease may develop due to reactivation of the measles virus or an inappropriate immune response to the measles virus. SSPE usually develops 2 to 10 years after the original viral attack. Initial symptoms may include memory loss, irritability, seizures, involuntary muscle movements, and/or behavioral changes, leading to neurological deterioration.

Symptoms

Subacute sclerosing panencephalitis is a rare neurological disease of childhood or young adulthood. The first signs are usually behavioral changes such as failing schoolwork, memory loss, and/or irritability. Involuntary muscle movements (myoclonic jerks) and generalized seizures follow. Subacute sclerosing panencephalitis is a progressive disease which results in personality changes, outbursts of temper, sleeplessness, disorientation, stupor, spasticity, loss of previously acquired intellectual skills, poor memory and judgment (dementia), and general neurological deterioration. Blindness may develop because of a lesion in the vision center of the brain (cortical blindness) and the nerves of the eyes may waste away (optic atrophy). The late symptoms of subacute sclerosing panencephalitis may include muscle rigidity, elevated body temperature (hyperthermia) and/or abnormalities of respiration, heartbeat, and blood pressure. These disturbances of normal bodily functions (homeostasis) indicate that the hypothalamus gland, which is located deep inside the brain, may be affected.



The complications of subacute sclerosing panencephalitis, such as severe pneumonia or coma, usually become life-threatening within 1 to 3 years. However, there may be improvement in some affected individuals for extended periods of time.

Causes

Subacute sclerosing panencephalitis is thought to be caused by a slow measles virus (paramyxovirus). Slow viruses may stay dormant in humans for extended periods of time, then for reasons yet unknown may become reactivated. The role of heredity which may make a person susceptible to slow viruses is not well understood.



The symptoms of SSPE, including inflammation of the brain (encephalitis) and the loss of the fatty covering on nerve fibers (demyelination), may develop due to reactivation of the virus many years after the initial illness. It may also be associated with an inappropriate immune response to the rubeola virus (measles). Typically, affected individuals have a history of measles infection 2 to 10 years before the onset of subacute sclerosing panencephalitis.



A few cases of subacute sclerosing panencephalitis in the medical literature have been associated with animal contact. These affected individuals had contact with pets such as monkeys, dogs, or kittens that later died of the same illness.

Affected Populations

With widespread uss of the measles vaccine in the United States, the incidence of subacute sclerosing panencephalitis has been reduced dramatically, although about 10 cases per year are reported. However, in less developed parts of the world, this disorder is much more common. In India, for example, the incidence is estimated at about 20 cases per year per million of population. Subacute sclerosing panencephalitis seems to affect males more often than females and occurs far more often in children and adolescents than in adults.

Standard Therapies

The diagnosis of subacute sclerosing panencephalitis may be confirmed by clinical evaluation and blood testing that reveals abnormally high levels of the measles antibody. Examination of the electrical activity of the brain (EEG) usually shows a characteristic pattern. The fluid surrounding the brain and spinal cord (cerebrospinal fluid) typically has elevated levels of gammaglobulin and measles antibody.



There is no specific treatment for subacute sclerosing panencephalitis. Anticonvulsants may help to control seizure activity. Other treatment is symptomatic and supportive.

Investigational Therapies

Several anti-viral agents, which have been investigated for the treatment of subacute sclerosing panencephalitis, have not been successful. Isolated reports about the effectiveness of the drug isoprinosine have not been confirmed by controlled clinical trials. More studies are needed to determine if the drug might be effective for some people under certain circumstances.



Other studies are ongoing for the treatment of subacute sclerosing panencephalitis. The drug interferon alpha (IFN), delivered directly into the spinal column (intrathecal) with and without the addition of oral inosiplex, is being tested for the treatment of this disease. Approximately 50 percent of affected individuals who had a slowly progressive form of the disease experienced an improvement in their symptoms with this treatment. More research is needed to determine the long-term safety and effectiveness of interferon alpha for the treatment of this disorder.



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

References

TEXTBOOKS

Dyken PR. Subacute Sclerosing Panencephalitis. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:574-75.



Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:2324-25.



Fauci AS, Braunwald E, Isselbacher KJ, et al. Eds. Harrison's Principles of Internal Medicine. 14th ed.McGraw-Hill Companies. New York, NY; 1998:2449.



Bernstein DI, Reuman PD, Schiff GM. Rubeola (Measles) and Subacute Sclerosing Panencephalitis. In: Gorbach SL, Bartlett JG, Blacklow NR. Eds. Infectious Diseases. W.B. Saunders Company, Philadelphia, PA; 1992:1754-58.



Adams RD, Victor M, Ropper AA. Eds. Principles of Neurology. 6th ed. McGraw-Hill Companies. New York, NY; 1997:767-68.



REVIEW ARTICLES

Garg RK. Subacute sclerosing panencephalitis. Postgrad Med J. 2002;78:63-70.



Dyken PR. Neuroprogressive disease of post-infectious origin: a review of resurging subacute sclerosing panencephalitis. Ment Retard Dev Disabil Res Rev. 2001;7:217-25.



JOURNAL ARTICLES

Alkan A, Sarac K, Kutlu R, et al. Early- and Late-State Subacute Sclerosing Panencephalitis: Chemical Shift Imaging and Single-Voxel MR Spectroscopy. ANJR Am J Neuroradiol. 2003;24:501-06.



Takashima H, Eriguchi M, Nakamura T, et al. Interferon therapy-reponsive brain meatbolic abnormalities in a case of adult-onset subacute sclerosing panencephalitis evaluated by 1H MRS analysis. J neurol Sci. 2003;207:59-63.



Kato Z, Saito K, Yamada M, et al. Proton magnestic resonsance spectroscopy in a case of subacute sclerosing panencephalitis. J Child Neurol. 2002;17:788-90.



Hayashi M, Arai N, Satoh J, et al. Neurodegenerative mechanisms in subacute sclerosing panencephalitis. J Child Neurol. 2002;17:725-30.



Solomon T, Hart CA, Vinjamuri S, et al. Treatment of subacute sclerosing panencephalitis with interferon-alpha, ribavirin, and Inosiplex. J Child Neurol. 2002;17:703-05.



Cruzado D, Masserey-Spicher V, Roux L, et al. Early onset and rapidly progressive subacute sclerosing panencephalitis after congenital measles infection. Eur J Pediatr. 2002;161:438-41.



Anlar B, Pinar A, Yasar Anlar F, et al. Viral studies in the cerebrospinal fluid in subacute sclerosing panencephalitis. J Infect. 2002;44:176-80.



FROM THE INTERNET

NINDS Subacute Sclerosing Panencephalitis Information page. Reviewed: 11-01-2002. 2pp.

www.ninds.nih.gov/health_and_medical/disorders/subacute_panencephalitis.htm



Restrepo L. Subacute sclerosing panencephalitis. MEDLINEplus. Medical Encyclopedia. Update Date: 8/3/2002. 3pp.

www.nlm.nih.gov/medlineplus/ency/article/001419.htm



Baumbach G. Parenchymal Infections: Subacute Sclerosing Panencephalitis. Virtual Hospital. nd. 2pp.

www.vh.org/adult/provider/pathology/CNSInfDisR2/Text/Pinf.VSSP.html



Appleton R. Subacute-Sclerosing Panencephalitis. Contact a Family. Last Updated: November, 2001. 2pp.

www.cafamily.orh.uk/Direct/s64.html



Turkington CA. Subacute sclerosing panencephalitis. HealthAtoZ. nd. 3pp.

www.healthatoz.com/healthatoz/Atoz/ency/subacute_sclerosing_penencephalitis.html



Subacute-Sclerosing Panencephalitis (SSPE). nd. 1p.

http://kobiljak.msu.edu/cai/pathology/CNS_Infectuions_F/CNS_1i.html



Subacute Sclerosing Panencephalitis (SSPE) & Vaccines. nd. 1p.

www.whale.to/vaccines/sspe1.html

Resources

Transverse Myelitis Association

1787 Sutter Parkway

Powell, OH 43065-8806

USA

Tel: (614)766-1806

Email: info@myelitis.org

Internet: http://www.myelitis.org



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Encephalitis Global

18 North Broadway #404

Tarrytown, NY 10591

USA

Tel: 604-980-2236

Fax: (604)904-0809

Email: admin@encephalitisglobal.org

Internet: http://www.encephalitisglobal.org



Encephalitis Society

32 Castlegate

Malton

North Yorkshire, YO17 7DT

United Kingdom

Tel: 4401653692583

Internet: http://www.encephalitis.info



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



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