Author/Editor		Rener-Primec, Z; Kopač, Š
Title		Parcialni napadi pri otrocih z Westovim sindromom
Translated title		Partial seizures in children with West syndrome
Type		članek
Source		Slov Pediatr
Vol. and No.		Letnik 5, št. Suppl 2
Publication year		1998
Volume		str. 123-6
Language		slo
Abstract		West sydrome(WS) is classified among the generalised epilepsies, however some patients also have partial seizures during the period of infantile spasms. The aim of this study was to establish the frequency and characteristics of partial seizures in children with West syndrome and their influence on the course of the illness. Methods: We retrospectively analysed the clinical, EEG and neuroradiological characteristics of 26 children, who were admitted to our Clinic for infantile spasms (IS) or WS in a 5-year period and who were at least 3 years of age during the period of follow-up. Resulst: Boys were more commonly affected with WS than girls (15:11). The cause of the illness was most frequently a perinatal hypoxic-ischaemic lesion of the central nervous system (8 patients), tuberous sclerosis (5 patients), cerebral malformation (3 patients), vascular lesion (1 patient) and metabolic lesion (1 patient). Cryptogenic IS were seen in 6 patients, idiopathic in 2 patients. Partial seizures occurred in the same period as the IS only in patients with symptomatic infantile plasms, whose outcomewas very poor; severe intellectual and motor retardation. Focal EEG signs were present in 18 patients, asymmetrical spasms and and focal neurological signs in 11 children. Focal EEG signs were consistent with CAT or MRI findings in 63% of patients. Conclusion: Cortical lesions can, in some patients with IS, cause both partial seizures and spasms, although in the majority of cases with West syndrome, any partial seizures occur shortly after the period of infantile spasms. The prognosis with regard to illness and development of these children is serious, most frrequently resulting in severe motor and intellectual retardation.
Summary		Westov sindrom (WS) je uvrščen v skupino generaliziranih epilepsij, vendar imajo nekateri bolniki v obdobju infantilnih spazmov tudi parcialne napade. Namen študije je ugoviti pogostnost pojavljanja in značilnosti parcialnih napadov pri otrocih z WS in vpliv na potek bolezni. Metode: Retrospektivno smo proučili klinične, elektroencefalografske (EEG) in nevroradiološke značilnosti 26 otrok, ki so bili sprejeti zaradi infantilnih spazmov (IS) oz. WS v 5-letnem obdobju na našo kliniko in so bili v času slednja stari 3 ali več let. Rezultati: WS je pogostejši pri dečkih kot pri deklicah (15:11). Vzrok bolezni je bila najpogosteje perinatalna hipoksično ishemična lezija osrednjega živčevja (8 bolnikov), tuberozna skleroza pri 5, malformacija možgan pri 3, vaskularna lezija pri enem in presnovna motnja pri enem otroku. Kriptogene infantilne splazme je imelo 6, idiopatske pa dva otroka. parcialne napade sočasno z obdobjem IS so imeli le bolniki s simptomatskimi infantilnimi splazmi, katerih izid bolezni je bil zelo slab: huda umska in motorična zaostalost. Žariščni znaki v EEG so bili prisotni pri 18 bolnikih, asimetrični spazmi ali žariščni nevrološki znaki pa pri 11 otrocih. Primerjava žariščnih znakov v EEG je bila v skladu z najdbo na slikovnih preiskavah (CT ali MRT) pri 63% bolnikov. Zaključek: Kortikalne lezije lahko pri nekaterih bolnikih z IS sprožijo tako parcialne napade kot splazme, seveda pa se pri večini bolnikov z Westovim sindromom, če se že pojavijo parcialni napadi, le-ti pojavijo kasneje, vendar kmalu po obdobju infantilnih splazmov. Prognoza glede bolezni in razvoja teh otrok je resna, saj največkrat vodi v hudo motorično in umsko manjrazvitost.
Descriptors		EPILEPSY, PARTIAL SPASMS, INFANTILE CHILD ELECTROENCEPHALOGRAPHY