Rasmussen's encephalitis

The Rasmussen encephalitis, even Rasmussen 's syndrome and in the English language called partly as chronic focal encephalitis (CFE ) is a named after Theodore Rasmussen progressive inflammation of parts of the cerebral cortex (cortex cerebri). The disease starts in a small area and then accesses the adjacent areas on a hemisphere. The opposite side of the brain is not usually affected. The inflammation leads to the loss of nerve cells with scarring and eventually leads to severe disability.

Epidemiology

The disease is usually diagnosed in children under 10 years, but can also occur during puberty or in adults. With an incidence of 1:500.000-1:1.000.000 it is one of the very rare diseases.

Clinical features

Usually, the first symptoms of the disease, focal epileptic seizures, neurological deficits such as hemiparesis later, visual disturbances ( hemianopsia ), speech disorders and cognitive disorders.

Cause

According to a study published in 2009, the disease is caused by a targeted attack on the immune system hitherto unknown structures in the brain. In this case, cytotoxic CD8-positive T- cell clones seem to attack the nerve cells and astrocytes. It is uncertain whether it is in the triggering structures are structures that belong to one's own body or possibly ingredients are as yet unknown viruses.

Treatment

In a series of studies different, also combined some of immunosuppressive treatments were tested, a clear treatment recommendations did not result so far. In advanced stages, brain surgery through to hemispherectomy can be made. Concomitant antiepileptic treatment is often difficult and has no effect on the progression of causal encephalitis.