Subacute Sclerosing Panencephalitis

What is Subacute Sclerosing Panencephalitis?

Subacute Sclerosing Panencephalitis (SSPE) is a rare, debilitating and ultimately fatal brain disorder that is caused by a mutation of the measles virus. It usually develops 7–10 years after a person has had the measles, and affects mostly young children between the ages of 2 and 12. While it is a rare disease, it can have devastating consequences for affected individuals and their families.

Although SSPE can be difficult to diagnose, it is important to recognize the warning signs as soon as possible to allow for early treatment and preparation for a future of likely disability. Once the symptoms appear, SSPE can progress rapidly, leading to severe mental and physical deterioration and eventually death.

Signs & Symptoms of SSPE

Symptoms of SSPE tend to surface gradually, usually starting at 6 months or more after initial measles infection. Common symptoms include:

  • Irritability
  • Lethargy
  • Depression
  • Changes in behavior
  • Decreased coordination
  • Slowed intellectual development
  • Seizures
  • Incontinence
  • Difficulty with speech

Diagnosis of SSPE

Diagnosing SSPE can be challenging because initial symptoms resemble those of many other illnesses. Diagnosis is based on a combination of patient history, neurological examination, EEG readings and laboratory tests, such as MRI or CT scans. It is possible to confirm SSPE with a test that looks for viral particles in spinal fluid.

Treatment of SSPE

Unfortunately, there is no known cure for SSPE and the disease is often difficult to treat. Treatment options have been limited to drugs that can slow down the progression of the disease, such as interferon-beta or other experimental drugs. These treatments have only been partially successful in the past and are usually only beneficial if they are started early on in the progression of the disease.

In addition to drug treatments, supportive care for SSPE can include physical therapy, occupational therapy, speech/language therapy, and psychotherapy. Dietary modifications, behavior management, environment modifications, and alternative therapies such as music and art may also be helpful.