West Syndrome

West Syndrome

Ndi chiyani ?

West syndrome, yomwe imatchedwanso kuti infantile spasms, ndi mtundu wosowa wa khunyu mwa makanda ndi ana omwe amayamba m'chaka choyamba cha moyo, nthawi zambiri pakati pa miyezi 4 ndi 8. Iwo yodziwika ndi spasms, kumangidwa kapena ngakhale regression wa psychomotor chitukuko cha mwana ndi matenda ubongo ntchito. Zomwe zimayambitsa matendawa zimakhala zosiyana kwambiri ndipo zimadalira zomwe zimayambitsa spasms, zomwe zingakhale zambiri. Zitha kuyambitsa zovuta zamagalimoto ndi luntha ndikupita ku mitundu ina ya khunyu.

zizindikiro

Ma Spasm ndi zizindikiro zoyamba za matendawa, ngakhale kuti kusintha kwa khalidwe la mwanayo kungakhale koyambirira. Nthawi zambiri zimachitika pakati pa miyezi 3 ndi 8, koma nthawi zina matendawa amatha kukhala oyambirira kapena mtsogolo. Kukomoka kwakanthawi kochepa kwambiri kwa minofu (sekondi imodzi kapena iŵiri) kodzipatula, nthawi zambiri mukadzuka kapena mukatha kudya, pang'onopang'ono kumayambitsa kuphulika kwa minyewa komwe kumatha kwa mphindi 20. Nthawi zina maso amatembenuzidwira m'mbuyo panthawi yomwe wagwidwa.

Spasm ndizizindikiro zowoneka chabe za kusokonekera kosatha muzochitika zaubongo zomwe zimawononga, zomwe zimapangitsa kuti kuchedwetsa kukukula kwa psychomotor. Chifukwa chake, mawonekedwe a spasms amatsagana ndi kuyimitsidwa kapena kuchepa kwa mphamvu zama psychomotor zomwe zapezeka kale: kuyanjana monga kumwetulira, kugwira komanso kusintha zinthu ...

Chiyambi cha matendawa

Ma Spasm amayamba chifukwa cha kulephera kwa ma neuron kutulutsa kutulutsa kwadzidzidzi komanso kosakhazikika kwamagetsi. Mavuto ambiri omwe amayamba chifukwa cha matenda a West syndrome amatha kudziwika mwa ana atatu mwa anayi omwe akhudzidwa: kupwetekedwa mtima, kusokonezeka kwa ubongo, matenda, matenda a kagayidwe kachakudya, matenda a chibadwa (Down syndrome, mwachitsanzo), matenda a neuro-cutaneous (mwachitsanzo, neuro-cutaneous disorders). matenda a Bourneville). Chotsatiracho ndi vuto lofala kwambiri lomwe limayambitsa West syndrome. Milandu yotsalayo imanenedwa kuti ndi "idiopathic" chifukwa imachitika popanda chifukwa, kapena "cryptogenic", ndiko kunena kuti mwina imalumikizidwa ndi vuto lomwe sitikudziwa momwe tingadziwire.

Zowopsa

West's syndrome sipatsirana. Zimakhudza anyamata pafupipafupi kuposa atsikana. Zili choncho chifukwa chimodzi mwa zifukwa zimene zimayambitsa matendawa n’zogwirizana ndi vuto la majini limene limakhudzana ndi X chromosome yomwe imakhudza amuna nthawi zambiri kuposa akazi.

Kupewa ndi chithandizo chamankhwala

Matendawa sangathe kudziwika zizindikiro zoyamba zisanayambe. Chithandizo chokhazikika ndikumwa mankhwala oletsa khunyu pakamwa tsiku lililonse (Vigabatrin nthawi zambiri amaperekedwa). Ikhoza kuphatikizidwa ndi corticosteroids. Opaleshoni imatha kulowererapo, koma mwapadera kwambiri, pamene matendawa alumikizidwa ndi zotupa zaubongo, kuchotsedwa kwawo kumatha kusintha mkhalidwe wa mwanayo.

Matendawa amasiyana kwambiri ndipo amatengera zomwe zimayambitsa matendawa. Zili bwino pamene mwana wakhanda ali wokalamba pa nthawi ya kuyambika kwa spasms yoyamba, mankhwala ndi oyambirira ndipo syndrome ndi idiopathic kapena cryptogenic. 80% ya ana okhudzidwa amakhala ndi zotsatira zomwe nthawi zina zimakhala zosasinthika komanso zovuta kwambiri: kusokonezeka kwa psychomotor (kuchedwa kulankhula, kuyenda, ndi zina zotero) ndi khalidwe (kusiya kudzikonda, kuchita zinthu mopitirira muyeso, kusowa chidwi, etc.). (1) Ana omwe ali ndi matenda a West syndrome nthawi zambiri amatha kudwala khunyu, monga matenda a Lennox-Gastaut (SLG).

Siyani Mumakonda