Rasmussen's syndrome
Rasmussen's syndrome is a progressive central nervous system disorder, characterized by
seizures, hemiparesis (paralysis on one side), inflammation of the brain, and mental
deterioration. This disorder occurs almost exclusively in children under the age of 10. Since
this disease was first given its name in 1958, the number of cases recorded in medical
literature is small. The cause is unknown, although it is thought to result from an unidentified
viral infection.
* very rare
* age of onset: (18 months to 14 years); <10 years in 80%
* focal motor fits or generalized tonic clonic fits
* intellectual deterioration, progressive hemiparesis
* variable severity, eventually plateaus, frequently leaves a neurologically devastated individual
* EEG shows bilateral or multifocal abnormalities with a unilateral predominance
* MRI shows progressive unilateral hemispheric atrophy beginning in the perisylvian region
? Ictal SPECT shows hyper-perfusion in the affected hemisphere
? Neuropathology: perivascular lymphocytic cuffing, glial nodules, microglial proliferation
Antibody to GluR3 is one pathogenetic mechanism
(
geocities.com/yale_er_doc)