Subacute Sclerosing Panencephalitis
Information Page |
NINDS is part of the
National Institutes of
Health
Table of Contents (click to jump to sections)
What is Subacute Sclerosing Panencephalitis?
Is there any treatment?
What is the prognosis?
What research is being done?
Organizations
Related NINDS Publications and Information
What is Subacute Sclerosing Panencephalitis?
Subacute sclerosing panencephalitis (SSPE) is a chronic persistent infection of the central nervous system caused by an altered
form of the measles virus. It affects primarily children and young adults and usually has a progressive downhill course which
results in death within a few years in most patients with a 5% chance of spontaneous remission. It can occur anywhere from
2 to 10 years after the original measles illness, and generally results in progressive neurological deterioration due to brain
inflammation and nerve cell death. Since the widespread use of the measles vaccine, SSPE has become very rare. However, studies
have shown that the incidence of SSPE has remained high in the Middle East and India. Initial symptoms usually include abnormal
behavior, irritability, intellectual deterioration, and memory loss which may be followed by involuntary movements and seizures
(in the form of myoclonic spasms). Subsequently, the patient develops further mental deterioration, inability to walk, speech
impairment with poor comprehension, and difficulty swallowing (dysphagia). Blindness may also result. In the final stages
of disease, the patient may remain mute or comatose. The electrical activity of the brain, as recorded by electroencephalogram
(EEG), shows progressive changes during the disease which are typical of SSEP and parallel the slow deterioration of central
nervous system functions. A number of clinical staging scales have been used for several decades to categorize patients with
SSPE according to their corresponding clinical status. More recently, a different staging system was developed based on the
radiological findings of the brain by computed tomography (CT) and magnetic resonance imaging (MRI). This method, however,
has not succeeded in establishing a complete correlation between radiological abnormalities and clinical progress.
Is there any treatment?
What is the prognosis?
What research is being done?
Select this link to view a list of studies currently seeking patients.
National Organization for Rare Disorders (NORD) P.O. Box 1968 (55 Kenosia Avenue) Danbury, CT 06813-1968 orphan@rarediseases.org http://www.rarediseases.org Tel: 203-744-0100 Voice Mail 800-999-NORD (6673) Fax: 203-798-2291 |
Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892
NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.
All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.
Last updated November 13, 2007