Rasmussen's encephalitis
| Rasmussen's encephalitis | |
|---|---|
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| Brain CT scan of a girl with Rasmussen's encephalitis. | |
| Classification and external resources | |
| Specialty | neurology |
| ICD-10 | G04.8 |
| ICD-9-CM | 323.81 |
| DiseasesDB | 33757 |
Rasmussen's encephalitis, also known as chronic focal encephalitis (CFE), is a rare inflammatory neurological disease, characterized by frequent and severe seizures, loss of motor skills and speech, hemiparesis (paralysis on one side of the body), encephalitis (inflammation of the brain), and dementia. The illness affects a single cerebral hemisphere and generally occurs in children under the age of 15.
The condition mostly affects children, with an average age of 6 years. However, one in ten people with the condition develops it in adulthood.
There are two main stages, sometimes preceded by a 'prodromal stage' of a few months. In the acute stage, lasting four to eight months, the inflammation is active and the symptoms become progressively worse. These include weakness of one side of the body (hemiparesis), loss of vision for one side of the visual field (hemianopia), and cognitive difficulties (affecting learning, memory or language, for example). Epileptic seizures are also a major part of the illness, although these are often partial. Focal motor seizures or epilepsia partialis continua are particularly common, and may be very difficult to control with drugs.
In the chronic or residual stage, the inflammation is no longer active, but the sufferer is left with some or all of the symptoms because of the damage that the inflammation has caused. In the long term, most patients are left with some epilepsy, paralysis and cognitive problems, but the severity varies considerably.
In Rasmussen’s encephalitis, there is chronic inflammation of the brain, with infiltration of T lymphocytes into the brain tissue. In most cases, this affects only one cerebral hemisphere, either the left or the right. This inflammation causes permanent damage to the cells of the brain, leading to atrophy of the hemisphere; the epilepsy that this causes may itself contribute to the brain damage. The epilepsy might derive from a disturbed GABA release, the main inhibitory neurotransmitter of the mammalian brain.
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