Subacute Sclerosing Panencephalitis: Causes, Symptoms, Treatment, Prognosis

Subacute Sclerosing Panencephalitis is an extremely rare but extremely serious and potentially fatal disorder of the brain which is basically caused by an abnormal reaction of the immune system of the body to the measles virus.

This abnormal immune response tends to cause irritation, swelling, and inflammation in the brain causing significant damage to the structure of the brain. This condition tends to occur years after recovering from measles.

Subacute Sclerosing Panencephalitis is very rare in the United States as most of the children here have been vaccinated against the measles virus. This condition usually affects children and young adults. It seems to occur more in males than in females.

Although Subacute Sclerosing Panencephalitis may be very rare in the United States, there are many places around the globe where there is less awareness about measles and not many children have been vaccinated against measles virus which puts them at increased risk for developing Subacute Sclerosing Panencephalitis.

It is not necessary that if an individual gets measles then that individual will develop Subacute Sclerosing Panencephalitis. There have been cases where children with measles have never developed Subacute Sclerosing Panencephalitis but it definitely puts an individual at risk for developing this condition which makes vaccination against measles that much more necessary in order to prevent Subacute Sclerosing Panencephalitis.

The exact cause for Subacute Sclerosing Panencephalitis is still under investigation but the most probable cause for development of this disease is believed to be infection of the brain as a result of measles virus.

This virus tends to infiltrate the brain during a measles infection. This results in the immune system getting activated causing inflammation in the brain and other symptoms consistent with encephalitis.

In some cases, the virus may infiltrate the brain but may stay dormant and not cause any symptoms for many years. A reactivation of this virus is believed to cause Subacute Sclerosing Panencephalitis. Studies suggest that in United States about 1 in a million people are diagnosed with Subacute Sclerosing Panencephalitis.

Some of the symptoms of Subacute Sclerosing Panencephalitis are:

• Behavioral changes occurring gradually over a span of time
• Decreasing cognitive skills
• Decreased social interaction
• Problems with completing tasks on time at home or at school
• Memory problems
• Fatigue
• Muscle spasms
• Weakness of the lower extremities
• Gait abnormalities
• Seizures
• Coma in advanced cases.

In order to diagnose Subacute Sclerosing Panencephalitis, the physician will first take a history of the patient and then check for the following signs which may point towards a diagnosis of Subacute Sclerosing Panencephalitis. These signs are:

• Checking the eyes to look for any damage to the retina or the optic nerve
• Testing the muscles to see for any signs of muscle twitching
• The physician will also check for coordination and movement of the child and in most of the cases of Subacute Sclerosing Panencephalitis the child will have very poor movement and coordination skills.

Additionally an EEG, MRI of the brain, and a spinal tap may be performed along with serum antibody titer to see if the child had a previous measles infection which may have been left undiagnosed. All of these tests will confirmatively diagnose Subacute Sclerosing Panencephalitis.

As of now, there is no treatment available for Subacute Sclerosing Panencephalitis. Antiviral medications may be given for slowing down the progression of the disease process. Seizures caused by Subacute Sclerosing Panencephalitis may be controlled to some extent by administering anticonvulsants. Apart from this, treatment is only symptomatic and supportive for Subacute Sclerosing Panencephalitis.

The overall prognosis for patients with Subacute Sclerosing Panencephalitis is extremely poor with majority of the patients succumbing to the disease within one to two years of the diagnosis of Subacute Sclerosing Panencephalitis.

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