West syndrome, tseem hu ua infantile spasms, yog ib hom mob qaug dab peg hauv cov menyuam mos thiab menyuam yaus uas pib hauv thawj xyoo ntawm lub neej, feem ntau ntawm 4 thiab 8 lub hlis. Nws yog tus cwj pwm los ntawm spasms, ntes lossis txawm tias regression ntawm psychomotor txoj kev loj hlob ntawm tus me nyuam thiab kev ua haujlwm tsis zoo ntawm lub hlwb. Qhov kev kwv yees yog qhov sib txawv heev thiab nyob ntawm qhov tshwm sim ntawm qhov spasms, uas tuaj yeem ua ntau yam. Nws tuaj yeem ua rau lub cev muaj zog thiab kev txawj ntse sequelae thiab nce mus rau lwm hom mob vwm.

Spasms yog thawj qhov tshwm sim ntawm tus mob syndrome, txawm hais tias tus me nyuam tus cwj pwm hloov yuav tau ua ntej lawv sai. Lawv feem ntau tshwm sim ntawm 3 thiab 8 lub hlis, tab sis qee zaus tus kab mob tuaj yeem ua ntej lossis tom qab. Cov leeg nqaij luv luv (ib mus rau ob vib nas this) cais, feem ntau thaum sawv ntxov lossis tom qab noj mov, maj mam muab txoj hauv kev rau kev tawg ntawm spasms uas tuaj yeem kav ntev li 20 feeb. Lub qhov muag qee zaum dov rov qab thaum lub sijhawm qaug dab peg.

Spasms tsuas yog pom cov cim qhia txog kev ua haujlwm tas mus li hauv lub hlwb kev ua haujlwm uas ua rau nws puas tsuaj, uas ua rau ncua kev txhim kho psychomotor. Yog li, cov tsos mob ntawm spasms yog nrog los ntawm stagnation lossis txawm tias qhov kev hloov pauv ntawm lub peev xwm psychomotor twb tau txais: kev sib cuam tshuam xws li luag nyav, tuav thiab tswj cov khoom ...

Spasms yog vim kev ua txhaum ntawm cov neurons tso tawm tam sim ntawd thiab txawv txav hluav taws xob. Ntau yam kab mob hauv qab tuaj yeem ua rau West Syndrome thiab tuaj yeem txheeb xyuas tsawg kawg peb lub hlis twg ntawm cov menyuam yaus cuam tshuam: kev yug me nyuam, lub hlwb malformation, kab mob, kab mob metabolic, genetic defect (down syndrome, piv txwv li), neuro-cutaneous disorders ( Bourneville tus kab mob). Qhov tom kawg yog qhov teeb meem feem ntau ua rau West syndrome. Cov xwm txheej ntxiv tau hais tias yog "idiopathic" vim tias lawv tshwm sim tsis muaj laj thawj, lossis "cryptogenic", uas yog hais tias tej zaum txuas rau qhov tsis xws luag uas peb tsis paub yuav txiav txim siab li cas.

West's syndrome tsis kis tau. Nws cuam tshuam cov tub hluas me ntsis ntau dua li cov ntxhais. Qhov no yog vim tias ib qho ntawm cov kab mob ua rau muaj kev cuam tshuam ntawm caj ces txuas nrog X chromosome cuam tshuam rau txiv neej ntau dua li poj niam.

Tus kab mob tsis tuaj yeem kuaj pom ua ntej thawj cov tsos mob tshwm sim. Cov txheej txheem kho mob yog noj tshuaj tiv thaiv kab mob vwm los ntawm qhov ncauj txhua hnub (Vigabatrin feem ntau yog tshuaj). Nws tuaj yeem ua ke nrog corticosteroids. Kev phais tuaj yeem cuam tshuam, tab sis tshwj xeeb tshaj yog, thaum tus mob no txuas nrog cov kab mob hauv lub paj hlwb, kev tshem tawm tuaj yeem txhim kho tus menyuam tus mob.

Qhov kev tshwm sim yog qhov sib txawv heev thiab nyob ntawm qhov ua rau ntawm tus mob syndrome. Nws yog txhua yam zoo dua thaum tus me nyuam mos laus thaum lub sij hawm pib ntawm thawj spasms, kev kho mob yog ntxov thiab cov syndrome yog idiopathic los yog cryptogenic. 80% ntawm cov menyuam yaus muaj kev cuam tshuam uas qee zaum hloov tsis tau thiab ntau dua lossis tsawg dua: kev puas siab puas ntsws (kev hais lus qeeb, taug kev, thiab lwm yam). (1) Cov menyuam yaus uas muaj West syndrome feem ntau ua rau muaj kab mob vwm, xws li Lennox-Gastaut syndrome (SLG).