Subacute sclerosing panencephalitis is caused by the measles virus.
The first symptoms are usually poor school performance, forgetfulness, temper outbursts, distractibility, sleeplessness, and hallucinations.
The diagnosis is based on symptoms.
This disorder is usually fatal.
There is no treatment for subacute sclerosing panencephalitis.
Subacute sclerosing panencephalitis results from a long-term brain infection with the measles Measles Measles is a highly contagious viral infection that causes various cold-like symptoms and a characteristic rash. Measles is caused by a virus. Symptoms include fever, runny nose, hacking cough... read more virus. The virus sometimes enters the brain during a measles infection. Measles virus may cause immediate symptoms of brain infection (encephalitis Encephalitis Encephalitis is inflammation of the brain that occurs when a virus directly infects the brain or when a virus, vaccine, or something else triggers inflammation. The spinal cord may also be involved... read more ), or the virus may remain in the brain for a long time without causing problems.
Subacute sclerosing panencephalitis occurs because the measles virus reactivates. In the past in the United States, for reasons that are not known, the disorder occurred in about 7 to 300 people per million people who had measles infection and in about 1 person per million people who received the measles vaccine. However, doctors think the people who developed subacute sclerosing panencephalitis after vaccination likely had a mild, undiagnosed case of measles before they were vaccinated and that the vaccine did not cause the subacute sclerosing panencephalitis.
Subacute sclerosing panencephalitis is rare in the United States and Western Europe because of widespread measles vaccination. However, analyses of more recent measles outbreaks suggest that the incidence of subacute sclerosing panencephalitis may be higher than previously thought.
Males are affected more often than females. The risk of developing subacute sclerosing panencephalitis is highest in people who contract measles before they are 2 years of age. Subacute sclerosing panencephalitis usually begins in children or young adults, usually before age 20.
Symptoms of SSPE
The first symptoms of subacute sclerosing panencephalitis may be poor performance in schoolwork, forgetfulness, temper outbursts, distractibility, sleeplessness, and hallucinations. Sudden muscular jerks of the arms, head, or body may occur. Eventually, seizures Seizure Disorders In seizure disorders, the brain's electrical activity is periodically disturbed, resulting in some degree of temporary brain dysfunction. Many people have unusual sensations just before a seizure... read more may occur, together with abnormal uncontrollable muscle movements. Intellect and speech continue to deteriorate.
Later, the muscles become increasingly rigid, and swallowing may become difficult. The swallowing difficulty sometimes causes people to choke on their saliva, resulting in pneumonia. People may become blind. In the final phases, the body temperature may rise, and the blood pressure and pulse become abnormal.
Diagnosis of SSPE
Tests on cerebrospinal fluid or blood
A doctor suspects subacute sclerosing panencephalitis in young people who have mental deterioration and muscle jerks and a previous history of measles. The diagnosis may be confirmed by examination of cerebrospinal fluid, a blood test that reveals high levels of antibody to the measles virus, by an abnormal electroencephalogram (EEG Electroencephalography Diagnostic procedures may be needed to confirm a diagnosis suggested by the medical history and neurologic examination. Electroencephalography (EEG) is a simple, painless procedure in which... read more ), and by magnetic resonance imaging (MRI Magnetic Resonance Imaging (MRI) In magnetic resonance imaging (MRI), a strong magnetic field and very high frequency radio waves are used to produce highly detailed images. MRI does not use x-rays and is usually very safe... read more ) or computed tomography (CT Computed Tomography (CT) In computed tomography (CT), which used to be called computed axial tomography (CAT), an x-ray source and x-ray detector rotate around a person. In modern scanners, the x-ray detector usually... read more ) that shows brain abnormalities.
A biopsy of the brain may need to be done if the tests cannot reveal a cause.
Prognosis for SSPE
Subacute sclerosing panencephalitis is nearly always fatal within 1 to 3 years. Although the cause of death is usually pneumonia, the pneumonia results from the extreme weakness and abnormal muscle control caused by the disease.
Treatment of SSPE
Antiseizure drugs for seizures
Nothing can be done to stop the progression of subacute sclerosing panencephalitis. Antiseizure drugs Antiseizure drugs In seizure disorders, the brain's electrical activity is periodically disturbed, resulting in some degree of temporary brain dysfunction. Many people have unusual sensations just before a seizure... read more may be taken to control or reduce seizures.