Subacute Sclerosing Panencephalitis
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What is Subacute Sclerosing Panencephalitis?Is there any treatment?What is the prognosis?What research is being done?OrganizationsWhat 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?
For several decades, the palliative treatment for SSPE has included anticonvulsant therapy and supportive measures (tube feedings
and good nursing care especially in patients with advanced disease). Medical literature during the last decade, however, has
shown stabilization of disease and delay in clinical progression after therapy with inosine pranobex (oral Isoprinosine);
oral isoprinosine combined with intrathecal or intraventricular interferon alpha (up to 50% rate of remission or improvement);
oral isoprinosine combined with interferon beta; and intrathecal interferon alpha combined with I.V. ribavirin. However, no
controlled studies have been performed. The Food and Drug Administration has added inosine pranobex (Isoprinosine) to its
List of Orphan Products Designations and Approvals (1988) for the treatment of SSPE.
What is the prognosis?
When not treated with immunomodulators (interferon) and antivirals (ribavirin and inosine pranobex) SSPE is almost always
a fatal disease. Death usually occurs between 1 and 3 years after onset, although some spontaneous remissions (up to 5%) have
been reported.
What research is being done?
The NINDS supports research on infections and diseases of the brain and nervous system including SSPE. Much of this research
is aimed at learning more about the cause(s), prevention, and treatment of these disorders. Currently no clinical trials on
SSPE are being conducted at the NIH.
Select this link
to view a list of studies currently seeking patients.
Organizations
Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892
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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.
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Last updated July 13, 2007
