Rasmussen's syndrome in children

Rasmussenov sindrom (RS), još poznat i kao Rasmussenov encefalitis (RE), je progresivna, neurološka bolest koja uglavnom pogađa djecu. Počinje se manifestirati u prosjeku oko 6. godine života i karakterizirana je unihemisferalnom atrofijom mozga, epileptičkim napadajima, hemiparezom, kognitivnom deterioracijom i, ako je zahvaćena moždana polutka koja je dominantna za govor i jezik, disfazijom. Kreće iz, naizgled, punog zdravlja te se u roku od nekoliko mjeseci pogoršava do stanja izrazito frekventnih epileptičkih napadaja koji napreduju do epilepsiae partialis continuae (EPS) ili statusa epilepticusa koji su najčešće potpuno refraktorni na lijekove. Prvotna slabost jedne polovice tijela progedira u unilateralnu hemiplegiju i većina bolesnika završava s nemogućnošću samostalnog hoda te u kolicima ili vezani za krevet. Točan uzrok se ne zna, ali činjenica je da je RE upalna bolest u kojoj cerebralni upalni infiltrat čine predominantno T limfociti. RE se dijagnosticira na temelju kliničke slike i karakterističnog nalaza na MR-u, a u tome pomažu kriteriji koji su predloženi 2005. godine. Liječenje se sastoji od primjene antiepileptika, imunoterapije i kirurškog liječenja, hemisferektomije (HS). Od toga troje, jedino je HS učinkovita u suzbijanju tvrdokornih i na lijekove nezaustavljivih epileptičkih napadaja. Također, utječe i na progresiju bolesti te neuropsihološko propadanje. RS se može manifestirati manje karakterističnim oblicima, ali to je vrlo rijetko.Rasmussen's syndrome (RS), also known as Rasmussen's encephalitis (RE), is a progressive, neurological disease which mostly affects children. It starts to manifest at 6 years of age on average and it is characterised by unihemispheric brain atrophy, epileptic seizures, hemiparesis, cognitive deterioration and, if the language-dominant hemisphere is affected, dysphasia. It develops from, seemingly, full health and arises in the time span of a few months into a condition of extremely frequent seizures which progress to epilepsia partialis continua (EPC) or status epilepticus, which are most commonly completely refractory to medication. The original weakness of one side of the body progresses into unilateral hemiplegia and most of the patients end up unable to walk independently, and in a wheelchair or bedbound. The exact cause remains unknown, but it is a fact that RE is an inflammatory disease in which the cerebral inflammatory infiltrate is predominantly made of T cells. RE is diagnosed by clinical examination and characteristic MRI findings, and the criteria, which were proposed in 2005, are helpful in the diagnosis. The treatment consists of antiepileptic drugs, immunotherapy and surgery, hemispherectomy (HS). Out of those three, HS is the only one effective in suppressing obdurate and intractable seizures. Also, HS affects the disease progression and neuropsychological deterioration. RS can be manifested in less characteristic forms, but that is very rare