After a highly febrile episode lasting for 4 weeks in 1990, a ten-year
old boy developed focal sensorimotor seizures of the left hand, initi
ally with secondary generalisation. Despite antiepileptic therapy epil
epsia partialis continua (EPC) persisted involving the left arm. EEC s
howed an epileptic focus, CT was normal at that time. A progressive sp
astic hemiparesis of the left side developed. 7 years later right-side
d hemiatrophia cerebri and contralateral cerebellar atrophy were revea
led by MRI/MRSI corresponding with low perfusion on Tc-99m-HMPAO-SPECT
, which showed 3 regions (anterior-temporal, frontal and parietal righ
t) with relative hyperperfusion probably correlated to epileptic foci.
History, clinical course, perfusion study, MRI and EEG findings are c
onsistent with Rasmussen's encephalitis. A virus infection is assumed
but the etiology of this childhood disease is not known. Recent data s
upport autoimmunological aspects triggering EPC by GluR3 antibodies. P
ersisting seizures are best treated with functional hemispherectomy.