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- $Unique_ID{BRK03704}
- $Pretitle{}
- $Title{Encephalitis, Rasmussen's}
- $Subject{Encephalitis, Rasmussen's Chronic Localized Encephalitis Chronic
- Encephalitis and Epilepsy Epilepsy, Hemiplegia and Mental Retardation Subacute
- Sclerosing Panencephalitis (SSPE)}
- $Volume{}
- $Log{}
-
- Copyright (C) 1987, 1989 National Organization for Rare Disorders, Inc.
-
- 415:
- Encephalitis, Rasmussen's
-
- ** IMPORTANT **
- It is possible the main title of the article (Rasmussen's Encephalitis)
- is not the name you expected. Please check the SYNONYMS listing on the next
- page to find alternate names, disorder subdivisions, and related disorders
- covered by this article.
-
- Synonyms
-
- Chronic Localized Encephalitis
- Chronic Encephalitis and Epilepsy
- Epilepsy, Hemiplegia and Mental Retardation
-
- Information on the following diseases can be found in the Related
- Disorders section of this report:
-
- Subacute Sclerosing Panencephalitis (SSPE)
-
- General Discussion
-
- ** REMINDER **
- The information contained in the Rare Disease Database is provided for
- educational purposes only. It should not be used for diagnostic or treatment
- purposes. If you wish to obtain more information about this disorder, please
- contact your personal physician and/or the agencies listed in the "Resources
- section of this report.
-
- Rasmussen's Encephalitis is a rare central nervous system disorder
- characterized by chronic active encephalitis (inflammation of the brain) and
- epileptic seizures of varying degrees of severity. Progressive symptoms
- including paralysis (usually of one side of the body) and mental retardation
- may also occur. Although the exact cause of this disorder is not known, it
- is thought to result from an unidentified viral infection.
-
- Symptoms
-
- Some cases of Rasmussen's Encephalitis are thought to follow inflammations or
- viral infections such as influenza, measles, etc. Epileptic seizures,
- usually mild, may be limited to certain areas of the body and may gradually
- spread and/or increase in severity. Encephalitis may not be diagnosed until
- post-mortem brain tissue is examined. Researchers believe the encephalitis
- may cause development of the seizures. Additionally paralysis which is
- usually limited to one side of the body, and mental retardation, often begin
- during early childhood and tend to progress over time.
-
- Causes
-
- The exact cause of Rasmussen's Encephalitis is not known. Some researchers
- believe it may result from an unidentified viral infection or slow virus.
- Slow viruses may stay dormant in humans for extended periods of time, then
- for reasons yet unknown may unexplainably become reactivated. The role of
- heredity which may make a person susceptible to slow viruses is not well
- understood.
-
- Affected Population
-
- Rasmussen's Encephalitis affects males and females in equal numbers. It is a
- very rare neurological disorder. Less than thirty cases have been described
- in the medical literature since this disorder was first identified in 1958.
- Also, fifty patients were identified during surgery world wide as of the end
- of 1986. A few hundred cases may develop each year but may not be properly
- identified.
-
- Related Disorders
-
- Symptoms of the following disorder can be similar to Rasmussen's
- Encephalitis. Comparisons may be useful for a differential diagnosis:
-
- Subacute Sclerosing Panencephalitis (SSPE) is a progressive brain
- disorder usually occurring months to years after an attack of measles. It is
- characterized by mental deterioration, involuntary jerky movements, and
- seizures. This disorder usually begins before the age of twenty, and may
- first be noticed by deteriorating schoolwork and sluggishness.
- Forgetfulness, temper outbursts, distractibility, sleeplessness,
- hallucinations, sudden flexion movements of the extremities or head and
- trunk, and grand mal seizures may follow the behavioral changes. Symptoms
- often progress in severity. Possible complications include coma. In rare
- cases, some patients may have remissions. (For more information on Subacute
- Sclerosing Panencephalitis, choose "SSPE" as your search term in the Rare
- Disease Database).
-
- Therapies: Standard
-
- Treatment of Rasmussen's Encephalitis is symptomatic and supportive. Social
- service agencies may be able to assist mentally retarded individuals and
- their families. Various medications may be used for control of seizures.
-
- Therapies: Investigational
-
- Brain surgery, known as hemispherectomy, for treatment of cases of
- Rasmussen's Encephalitis is being tested. In general, the portion of the
- brain affected by encephalitis is removed in an attempt to control chronic
- epileptic seizures and progressive neurological degeneration. More research
- is necessary before this drastic surgery may prove of benefit to those
- affected by this disorder. For more information, please contact:
-
- Dr. Ben Carson
- Chief of Pediatric Neurosurgery
- Children's Center
- Johns Hopkins Hospital
- Baltimore, MD 21205
-
- This disease entry is based upon medical information available through
- September 1989. 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.
-
- Resources
-
- For more information on Rasmussen's Encephalitis, please contact:
-
- National Organization for Rare Disorders (NORD)
- P.O. Box 8923
- New Fairfield, CT 06812-1783
- (203) 746-6518
-
- NIH/National Institute of Neurological Disorders & Stroke (NINDS)
- 9000 Rockville Pike
- Bethesda, MD 20892
- (301) 496-5751
- (800) 352-9424
-
- Centers for Disease Control (CDC)
- 1600 Clifton Road, NE
- Atlanta, GA 30333
- (404) 639-3534
-
- Dr. Theodore Rasmussen
- Montreal Neurological Hospital
- 3801 University Street
- Montreal 112,
- Canada
- (514) 284-5804
-
- Dr. Ben Carson
- Chief of Pediatric Neurosurgery
- Children's Center
- Johns Hopkins Hospital
- Baltimore, MD 21205
-
- References
-
- FOCAL SEIZURES DUE TO CHRONIC LOCALIZED ENCEPHALITIS: T. Rasmussen, et al.;
- Neurology (June 1958, issue 8(6)). Pp. 435-445.
-
- FURTHER OBSERVATIONS ON THE SYNDROME OF CHRONIC ENCEPHALITIS AND
- EPILEPSY: T. Rasmussen; Applied Neurophysiology (1978, issue 41). Pp. 1-
- 12.
-
- SMOLDERING ENCEPHALITIS IN CHILDREN: P.C. Gupta, et al.;
- Neuropediatrics (October 1984, issue 15(4)). Pp. 191-197.
-
-