I-chorea kaHuntington iyisifo esizalisayo esisodwa, esihambisana nokunyakaza okungazibandakanyi, ukwehla kobuhlakani nokuthuthukiswa kwezifo ezingokomqondo. Lesi sifo singakhula emadodeni nakwabesifazane nakancane noma yikuphi ubudala, kodwa ngokuvamile ngokuvamile izimpawu zokuqala ze-chorea kaHuntington zivela eminyakeni yobudala engama-35-40.
Izimpawu zezifo zikaHuntington
Isibonakaliso esiyinhloko esibhedlela sesifo sikaHuntington yisifo se-chorea, esibonakaliswa ukunyakaza okuphazamisekile nokungalawulwa. Ekuqaleni, lokhu kungukuphazanyiswa okuncane kuphela ekuxhumaneni nokuhamba okunamandla kwezandla noma izinyawo. Lokhu ukuhamba kungase kube kancane noma kuphuthumayo. Ngokuhamba kancane, bamba umzimba wonke futhi bahlale bodwa, badle noma bagqoke kuba nzima. Ngemuva kwalokho, ezinye izimpawu zesifo sikaHuntington ziqala ukunamathela kuloluphawu:
- izinkinga ngenqubo yokuhlafuna nokugwinya;
- ukukhubazeka kwenkulumo;
- izinkinga zokulala;
- ukwephulwa komsindo we-muscle.
Ngesikhathi sokuqala, kungenzeka kube nezinkinga ezincane zobuntu nemisebenzi yokuqonda. Isibonelo, isiguli sinokwephulwa kwemisebenzi yokucabanga okungabonakali. Ngenxa yalokho, akakwazi ukuhlela izenzo, azenze futhi azinikeze ukuhlolwa okwanele. Khona-ke lezi zinkinga ziba nzima kakhulu: umuntu uba nobudlova, ahlukumeze ngokocansi, azibophezele yena, imibono ekhukhumezayo ibonakale futhi umlutha (ukuphuza ngokweqile, ukugembula) kwanda.
Ukuxilongwa kwezifo zikaHuntington
Ukuxilongwa kwe-Huntington's syndrome kwenziwa ngezindlela ezehlukene zokuhlolwa kwengqondo nokuhlolwa ngokomzimba. Phakathi kwezinyathelo eziyinhloko, indawo eyinhloko ihlala ngokucabangela kwamagnetic resonance kanye ne-tomography ehlanganisiwe. Kungenxa ngosizo lwabo ukuthi ungabona indawo yokulimala kwengqondo.
Ukuhlolwa kofuzo kusetshenziselwa izindlela zokuhlola. Uma kunezinhlayiya ezingaphezu kwezingu-38 ze-CAG ezitholakala ku-geni ye-HD, isifo sikaHuntington sizogcina sibe ngamacala angu-100%. Kulesi simo, ezincane inani lezinsalela, kamuva esikhathini esizayo impilo izobonakalisa i-chorea.
Ukwelashwa kwezifo zikaHuntington
Ngeshwa, isifo sikaHuntington asipheli. Okwamanje, empini yokulwa nalesi sifo, kusetshenziswa kuphela ukwelashwa okuyisimangaliso, okusiza okwesikhashana isimo sesiguli.
Isidakamizwa esiwusizo kakhulu, esibuthakathaka izimpawu zesifo, yiTetrabenazine. Futhi ekwelapheni kukhona izidakamizwa eziphikisana ne-Parkinsonian:
- I-Bromocriptine;
- Levodopa;
- I-Pergolid.
Ukuqeda i-hyperkinesia nokunciphisa ukuqina kwemisipha, i-valproic acid isetshenziswa. Ukwelashwa kokucindezeleka kule sifo kwenziwa nge-Prozac, i-Citalopram, i-Zoloft namanye ama-inhibitors ekhethayo we-serotonin reuptake. Uma kuthuthukiswa ingqondo, i-antipsychotics ye-atypical (Risperidone, Clozapine noma i-Amisulpride) isetshenziswa.
Isikhathi sokuphila kubantu abahluphekayo ngesifo sikaHuntington sinciphise kakhulu. Kusukela ngesikhathi sokubonakala kwezibonakaliso zokuqala zalesi sifo sokufa kungadlula iminyaka engu-15 kuphela. Ngesikhathi esifanayo, elibulalayo
- imizamo yokuzibulala;
- i-pneumonia;
- ukukhubazeka kwenhliziyo.
Ngenxa yokuthi lesi yisifo sofuzo, ukuvimbela ngokwayo akukhona. Kodwa kusukela ekusetshenzisweni kwezinqubo zokuhlola (ukuhlolwa kokubeletha ngaphambi kokuhlaziywa kwe-DNA) akudingekile ukuba unqabe, ngoba uma kuqala ezinyangeni zokuqala ukwelashwa okuphawulekayo, ungakwazi kakhulu ukukhulisa impilo yesiguli.