Subacute sclerosing panencephalitis is a progressive, usually fatal brain damage that occurs months to years after a measles disease. It leads to a loss of brain functions, myoclonic reflexes and seizures. The diagnosis is u. a. from the electroencephalogram, computed tomography, and cerebrospinal fluid Masernserologie. Treatment is supportive.
Subacute sclerosing panencephalitis (SSPE) probably represents (measles) persistent measles virus infection. The measles virus can be detected in the brain tissue.
Subacute sclerosing panencephalitis is a progressive, usually fatal brain damage that occurs months to years after a measles disease. It leads to a loss of brain functions, myoclonic reflexes and seizures. The diagnosis is u. a. from the electroencephalogram, computed tomography, and cerebrospinal fluid Masernserologie. Treatment is supportive. Subacute sclerosing panencephalitis (SSPE) probably represents (measles) persistent measles virus infection. The measles virus can be detected in the brain tissue. SSPE is approximately 7-300 per 1 million people with measles wild-type infection before, and at about 1 case per 1 million people vaccinated against measles; Some cases may have been caused by an unrecognized measles infection before vaccination. Men are more commonly affected. The disease usually begins before age 20. SSPE extremely rare occurrence in the US and Western Europe. Symptoms and complaints Often the first symptoms are discreet – poorer academic performance, forgetfulness, temper tantrums, distractibility and insomnia. However, it can then come in the course to hallucination and myoclonic reflexes, followed by generalized seizures. It’s still coming to mental decline and language disorders. Dystonic movements and a transient opisthotonus may occur. Later, it may cause muscle stiffness, difficulty swallowing, cortical blindness and optic atrophy. There is often a focal chorioretinitis and other Funduscopic abnormalities. In the final stage, it may cause an intermittent hyperthermia, diaphoresis, and pulse and blood pressure fluctuations due to involvement of the hypothalamus. Diagnosis Serological tests electroencephalogram Neuroimaging The suspected SSPE is in young patients with dementia and neuromuscular excitability. There are carried out an electroencephalogram, a computed tomography or magnetic tomographs, cerebrospinal fluid and a Masernserologie. In the electroencephalogram to find periodic complexes with diphasic waves of high voltage appearing synchronously over the entire recording time. In computed tomography or magnetic resonance imaging a cortical atrophy or white matter lesions may be visible. CSF examination usually gives standard values ??for intracranial pressure, cell counts and total protein; Liquorimmunglobuline, however, are almost always increased and make up to 20-60% of Liquorproteins out. In serum and CSF increased Masernvirusantikörpertiter are detectable. The anti-measles IgG appears to increase with advancing disease. With inadequate test results, a brain biopsy may be necessary. Prognosis The disease is almost always fatal within 1-3 years (often a pneumonia the cause of death), in some patients, the course is, however protracted. Few patients have remissions and exacerbations. Treatment Supportive treatment The only approved therapies are the administration of anticonvulsants and other supportive measures. The use of isoprinosine, interferon alpha and lamivudine is controversial, and antiviral agents have generally proved to be unhelpful.