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$Title{Subacute Sclerosing Panencephalitis}
$Subject{Subacute Sclerosing Panencephalitis SSPE Decerebrate Dementia }
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Copyright (C) 1986, 1987 National Organization for Rare Disorders, Inc.

185:
Subacute Sclerosing Panencephalitis

** IMPORTANT **
It is possible the main title of the article (Subacute Sclerosing
Panencephalitis) is not the name you expected.  Please check the SYNONYMS
listing to find the alternate names and disorder subdivisions covered by this
article.

Synonyms

     SSPE
     Decerebrate Dementia

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.


Subacute Sclerosing Panencephalitis (SSPE) is a progressive life
threatening neurological (brain) disorder occuring months to years (usually
years) after an attack of measles.  It is characterized by mental
deterioration, myoclonic (shocklike) jerks, and seizures.

Symptoms

Subacute Sclerosing Panencephalitis usually begins before the age of 20
years.  Often the first signs are failing schoolwork, forgetfulness, temper
outbursts, distractibility, sleeplessness, and hallucinations.  Myoclonic
jerks (sudden flexion movements of the extremities, head and trunk) and grand
mal seizures may follow the mental and behavioral changes.

Patients suffering from SSPE show further intellectual decline, changes
in speech and abnormal involuntary movements.  Distortion and twisting of the
body, head and extremities may appear temporarily.  Later, rigidity of the
body muscles, difficulty in swallowing, cortical blindness, and optic atrophy
may occur.  In the advanced phases of SSPE, the patient becomes increasingly
rigid, with intermittent signs of hypothalamic involvement (i.e., high body
temperature, profuse perspiration, and disturbance of pulse and blood
pressure).

The disease can become life threatening within 1 to 3 years, often as the
result of terminal bronchial pneumonia due to inactivity or aspiration of
food.  Sometimes, it has a more protracted course, with pronounced
neurological deficits.  A few patients may have remissions and exacerbations.

Causes

The cause of Subacute Sclerosing Panencephalitis is unknown; possibly SSPE is
caused by a virus.  Usually there has been a history of mumps or measles 2-10
years prior to the onset, but these childhood illnesses are common in the
general population.  There have been cases where patients have had contact
with pets such as monkeys, dogs or kittens which later have died from the
illness.

Affected Population

Subacute Sclerosing Panencephalitis occurs in children and adolescents
usually before the age of 20 years.

Related Disorders

Progressive Multifocal Leukoencephalopathy is an infection of the brain by a
normally nonpathogenic virus.  Symptoms include unilateral bodily weakness,
visual impairment and an alteration in the state of consciousness.

Inclusion Body Encephalitis is a brain infection with gradual onset
mostly in children under 12 years of age.  Symptoms include deterioration in
schoolwork, muscle jerks of the trunk and extremities, and loss of speech.

Therapies:  Standard

Generally, treatment of the symptoms of Subacute Sclerosing Panencephalitis
with anticonvulsants and supportive measures can be helpful.  A number of
antiviral agents have not proven helpful.  Isolated reports about the
effectiveness of isoprinosine have not been documented in controlled clinical
trials.

Therapies:  Investigational

This disease entry is based upon medical information available through March
1987.  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 Subacute Sclerosing Panencephalitis, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

     National SSPE Registry
     University of Alabama School of Medicine
     Department of Neurology
     2451 Fillingim St.
     Mobile, AL  36617
     (205) 471-2159

     NIH/National Institute of Allergy and Infectious Diseases (NIAID)
     9000 Rockville Pike
     Bethesda, MD  20892
     (301) 496-5717

References

THE MERCK MANUAL, 15th ed., Robert Berkow, M.D., ed in chief, published by
Merck, Sharp & Dohme Research Labs, Rahway, NJ, 1987.  Pp. 1401, 2023, 2041.

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W. B. Saunders Co., 1988.  Pp. 2203, 2206-7.