Subacute Sclerosing Panencephalitis

Caused by an altered form of the measles virus, subacute sclerosing panencephalitis is a chronic persistent infection of the central nervous system. Primarily affecting children and young adults, this condition usually has a progressive downward course that results in death within a few years for most patients. Since the widespread use of the measles vaccine, subacute sclerosing panencephalitis has become very rare in the United States.

 

An Overview of Subacute Sclerosing Panencephalitis

Subacute sclerosing panencephalitis is a chronic persistent infection of the central nervous system caused by an altered form of the measles virus.
 
Primarily affecting children and young adults, subacute sclerosing panencephalitis usually has a progressive downward course, which, for most patients, results in death within a few years; however, some cases of spontaneous remission (up to 5 percent) have been reported.
 
Subacute sclerosing panencephalitis can occur anywhere from 2 to 10 years after the original measles illness. Generally, subacute sclerosing panencephalitis results in progressive neurological deterioration due to brain inflammation and nerve cell death.
 
Since the widespread use of the measles vaccine, subacute sclerosing panencephalitis has become very rare. However, studies have shown that the incidence of subacute sclerosing panencephalitis has remained high in the Middle East and India.
 

Symptoms of Subacute Sclerosing Panencephalitis

Initial symptoms of subacute sclerosing panencephalitis usually include:
 
  • Abnormal behavior
  • Irritability
  • Intellectual deterioration
  • Memory loss
  • Involuntary movements
  • Seizures (in the form of myoclonic spasms).
     
Subsequently, the patient with subacute sclerosing panencephalitis can develop:
 
  • Further mental deterioration
  • Inability to walk
  • Speech impairment with poor comprehension
  • Difficulty swallowing (dysphagia).
  • Blindness.
     
In the final stages of subacute sclerosing panencephalitis, the patient may remain mute or comatose.
 
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Written by/reviewed by: Arthur Schoenstadt, MD
Last reviewed by: Arthur Schoenstadt, MD