West syndrome, inonziwo infantile spasms, inzira isingawanzoitiki yepfari muvacheche nevana inotanga mugore rekutanga rehupenyu, kazhinji pakati pemwedzi 4 ne8. Iyo inoratidzirwa ne spasms, kusungwa kana kunyange kudzoreredzwa kweiyo psychomotor kukura kwemucheche uye kusagadzikana kwehuropi basa. Chirevo chacho chinopesana zvikuru uye chinoenderana nezvikonzero zvinokonzera spasms, iyo inogona kuva yakawanda. Inogona kukonzera yakakomba mota uye yehungwaru sequelae uye kufambira mberi kune mamwe marudzi epfari.

Spasms ndiyo yekutanga kuratidzwa kunoshamisa kweiyo syndrome, kunyangwe maitiro akachinja emucheche angave akavatangira munguva pfupi. Zvinowanzoitika pakati pemwedzi mitatu ne3, asi muzviitiko zvisingawanzoitiki chirwere chinogona kunge chiri mberi kana kuti gare gare. Kupfupika kwetsandanyama kupfupi (sekondi imwe kusvika kumaviri) kwakaparadzaniswa, kazhinji pakumuka kana kuti mushure mekudya, zvishoma nezvishoma kunopa nzira kune kuputika kwezviputi zvinogona kugara kwemaminitsi makumi maviri. Maziso dzimwe nguva anotenderedzwa kumashure panguva yekubatwa.

Spasms zviratidzo zvinooneka zvekusashanda zvachose muhuropi chiitiko chinoikuvadza, zvichikonzera kunonoka kukura kwepfungwa. Saka, kutaridzika kwe spasms kunoperekedzwa nekumira kana kunyange kudzoreredzwa kwe psychomotor hunyanzvi hwatowanikwa: kusangana kwakadai sekunyemwerera, kubata uye kugadzirisa zvinhu ... Electroencephalography inoburitsa mafungu ehuropi ayo anonzi hypsarrhythmia.

Spasms imhaka yekutadza kuita kweneuroni kuburitsa kubuda kwemagetsi kamwe kamwe uye kusingaite. Zvinetso zvakawanda zvinogona kuva chikonzero cheWest syndrome uye zvinogona kuonekwa muzvikamu zvitatu kubva muzvina zvevana vakabatwa: kushungurudzika kwekuberekwa, kukanganisa kwehuropi, utachiona, chirwere chemagetsi, genetic defect (Down syndrome, somuenzaniso), neuro-cutaneous disorders. Bourneville chirwere). Iyo yekupedzisira ndiyo inowanzoitika chirwere chinokonzera West syndrome. Mhosva dzakasara dzinonzi "idiopathic" nekuti dzinoitika pasina chikonzero chakajeka, kana "cryptogenic", kureva kuti pamwe yakabatana neanomaly yatisingazivi nzira yekuziva.

West's syndrome haina kutapurirana. Inobata vakomana kakawanda zvishoma pane vasikana. Izvi zvinodaro nekuti chimwe chezvikonzero zvechirwere ichi chine chekuita nekuremara kwemajini kwakabatana neX chromosome inobata varume kakawanda kupfuura vakadzi.

Chirwere hachigoni kuonekwa zviratidzo zvekutanga zvisati zvaonekwa. Mushonga wakajairika ndewekutora mushonga unorwisa pfari nemuromo zuva nezuva (Vigabatrin inonyanya kunyorerwa). Inogona kusanganiswa ne corticosteroids. Kuvhiya kunogona kupindira, asi zvakanyanyisa, apo chirwere chacho chakabatanidzwa kune maronda europi emunharaunda, kubvisa kwavo kunogona kuvandudza mamiriro emwana.

Chirevo chacho chinosiyana zvikuru uye chinoenderana nezvikonzero zvinokonzera chirwere chacho. Izvo zvose zviri nani apo mucheche akwegura panguva yekutanga kwekutanga spasms, kurapwa kunotanga uye syndrome idiopathic kana cryptogenic. 80% yevana vanobatwa vane sequelae iyo dzimwe nguva isingachinjiki uye yakanyanya kana kuderera: kusagadzikana kwe psychomotor (kunonoka pakutaura, kufamba, nezvimwewo) uye maitiro (kuregera mukati mako, kukurumidza, kusatarisisa, nezvimwewo). (1) Vana vane West syndrome vanowanzobatwa nechirwere chepfari chinotevera, chakadai seLennox-Gastaut syndrome (SLG).