I-West syndrome, ebizwa nangokuthi i-infantile spasms, iwuhlobo olungavamile lwesithuthwane ezinsaneni nasezinganeni oluqala onyakeni wokuqala wokuphila, ngokuvamile phakathi kwezinyanga ezi-4 neziyisi-8 ubudala. Ibonakala ngama-spasms, ukuboshwa noma ukuhlehla kokuthuthukiswa kwe-psychomotor yengane kanye nomsebenzi wobuchopho obungavamile. I-prognosis iyahlukahluka kakhulu futhi incike ezimbangela eziyinhloko ze-spasms, ezingaba ziningi. Kungabangela i-motor eyingozi kanye ne-sequelae yobuhlakani futhi kuthuthukele kwezinye izinhlobo zesithuthwane.

I-Spasm iwukubonakaliswa kokuqala okuphawulekayo kwe-syndrome, nakuba ukuziphatha okushintshiwe kwengane kungenzeka ukuthi kwandulele maduzane. Ngokuvamile zenzeka phakathi kwezinyanga ezi-3 neziyi-8, kodwa ezimweni ezingavamile lesi sifo singase sibe ngaphambi kwesikhathi noma kamuva. Ukufinyela kwemisipha okufushane kakhulu (umzuzwana owodwa kuya kwemibili) kuhlukanisiwe, ngokuvamile lapho uvuka noma ngemva kokudla, kancane kancane kuthathela indawo ukuqhuma kwezinhlungu ezingahlala imizuzu engu-20. Amehlo kwesinye isikhathi ahlehliswe emuva ngesikhathi sokuquleka.

Ama-Spasm ayizimpawu ezibonakalayo kuphela zokungasebenzi kahle okungapheli emsebenzini wobuchopho okuwulimazayo, okuholela ekuthuthukisweni kwe-psychomotor ukubambezeleka. Ngakho-ke, ukubonakala kwama-spasms kuhambisana nokuma noma ukuhlehla kwamandla e-psychomotor asevele atholakele: ukusebenzisana okufana nokumomotheka, ukubamba kanye nokukhwabanisa kwezinto ... I-Electroencephalography yembula amaza obuchopho adidekile abizwa ngokuthi i-hypsarrhythmia.

Ama-Spasm abangelwa umsebenzi ongalungile wama-neuron akhipha ukuchitheka kukagesi kungazelelwe nokungajwayelekile. Iziyaluyalu eziningi ezicashile zingaba imbangela ye-West syndrome futhi zingabonakala okungenani ezingxenyeni ezintathu kwezine zezingane ezithintekile: ukuhlukumezeka kokuzalwa, ukukhubazeka kobuchopho, ukutheleleka, isifo se-metabolic, ukukhubazeka kofuzo (i-Down syndrome, isibonelo), ukuphazamiseka kwe-neuro-cutaneous (i-neuro-cutaneous disorders) Isifo sikaBourneville). Lesi sakamuva yisifo esivame kakhulu esibhekene ne-West syndrome. Amacala asele kuthiwa "yi-idiopathic" ngoba ayenzeka ngaphandle kwesizathu esicacile, noma "i-cryptogenic", okusho ukuthi mhlawumbe ixhunywe ku-anomaly esingazi ukuthi singanquma kanjani.

I-West's syndrome ayitheleleki. Sihlasela abafana kaningi kunamantombazane. Lokhu kungenxa yokuthi enye yezimbangela zalesi sifo ihlobene nenkinga yofuzo ehlobene ne-X chromosome ethinta amadoda kaningi kunabesifazane.

Lesi sifo asikwazi ukutholwa ngaphambi kokuba kuvele izimpawu zokuqala. Ukwelashwa okujwayelekile ukuthatha umuthi ovimbela isithuthwane ngomlomo nsuku zonke (iVigabatrin ivame ukunikezwa). Ingahlanganiswa ne-corticosteroids. Ukuhlinzwa kungangenelela, kodwa ngendlela engavamile, lapho i-syndrome ixhunyaniswa nezilonda zobuchopho zendawo, ukususwa kwazo kungathuthukisa isimo sengane.

I-prognosis iyahlukahluka kakhulu futhi incike kuzimbangela eziyinhloko ze-syndrome. Kungcono kakhulu lapho umntwana esekhulile ngesikhathi sokuqala kwe-spasms yokuqala, ukwelashwa kusenesikhathi futhi i-syndrome i-idiopathic noma i-cryptogenic. U-80% wezingane ezithintekile zinezimpendulo ngezinye izikhathi ezingahle zihlehliswe futhi ezingathí sina kakhulu: ukuphazamiseka kwengqondo (ukubambezeleka ekukhulumeni, ukuhamba, njll.) nokuziphatha (ukuzihoxisa, ukuxakeka ngokweqile, ukungakwazi ukunaka, njll.). (1) Izingane ezine-West syndrome zivame ukuthambekela ekubeni nesifo sokuwa esilandelayo, njenge-Lennox-Gastaut syndrome (SLG).