Werdnig-Hoffmann: ifomu kanye izimpawu eziyinhloko

Werdnig-Hoffmann - kakhulu zokugula sina okuyinto kuhambisana amyotrophy, ukubhujiswa kancane kancane main yezinzwa we umgogodla, futhi ngenxa yalokho, imisipha yoma. Ngokushesha kuyafaneleka ukuthi lena isifo ngofuzo zofuzo, okuyinto idluliselwa i autosomal ngendlela esikhubazayo.

Kuyini Werdnig-Hoffmann?

Yisifo zezinzwa ephelezelwa ukubhujiswa kancane kancane izakhiwo eziyisisekelo kwemizwa uhlelo innervating. Ngokwesibonelo, ngokuvamile kukhona demyelination izimpande anterior ka emgogodleni. Ngaphezu kwalokho, lesi sifo sihlasela ezinye izinzwa cranial.

Yiqiniso, umonakalo owenziwe izinzwa kuthinta isimo imisipha. Nokho, lesi sifo libhekene okuthiwa ugongolo imisipha ukulahlekelwa, lapho ingxenye imisipha unawo amandla ungenwa, kuyilapho ehlukile "izinyanda" yoma.

yoma Spinal Verdniga-Hoffmann ebonakaliswa ebuntwaneni. Namuhla ihlukaniswe izinhlobo ezintathu eziyinhloko zesifo.

yesifo azalwa naso Verdniga-Hoffmann kanye nezimpawu zayo

Ngokuvamile, izimpawu zokuqala yaleli fomu lesi sifo kakade olubonakalayo izinsuku zokuqala ezimbalwa emva kokuzalwa. Ingane ukwethula evilaphayo paresis wemikhawulo. Ukukhala izingane ezigulayo ababuthakathaka ngokulambisa ezwakalayo, ngaphezu, banayo inqubo amandla siphukile.

Njengoba ingane yakho ikhula, ungakwazi ukuqaphela lag ekuthuthukisweni ngokomzimba. izingane ezigulayo musa ukugcina ikhanda, abakwazi ukuhlala noma ume. Kuphela ezimweni ezingavamile, umntwana usuke esekwazi ukuqhuba umzimba sesimweni beqotho, kodwa leli khono ubalekela angabacwasi ngokushesha njengoba ukubhujiswa yezinzwa.

Ngaphezu kwalokho, kukhona ezinye kwezinso ekuthuthukisweni lwamathambo, dysplasia ikakhulukazi hip, ukugobeka komhlandla, ukwakheka uphondo esifubeni, kokukhubazeka, iphimbo.

Phakathi esibulalayo, futhi angakwazi kwalapho uyathuthuka ngokushesha. Yoma lingachazwa hhayi kuphela imisipha yohlaka, kodwa futhi Imicu yotshani izitho zangaphakathi. Ngokuvamile abathintekayo diaphragm, okuholela nokuvaleka kwemigudu yokuphefumula. Ngeshwa, abantwana nge isifo esifanayo siphile (ngokwesilinganiso) kuze kufinyelele eminyakeni ewu elithoba.

Early Verdniga-Hoffmann isifo

Izimpawu oyinhloko sifo azibonakali engxenyeni wokuphila wesibili. Phakathi nezinyanga zokuqala ezimbalwa ekuthuthukiseni ngokomzimba wengane sihamba ejwayelekile - kid ifunda ubambe ikhanda futhi ahlale, ngezinye izikhathi ngisho kungaba nokuhlala ungasizwa muntu. Kodwa zonke lezi amakhono elahleka emva kusebenze lesi sifo. By the way, ngokuvamile kuphakamisa ukutheleleka syndrome.

Eliqhaqhazelayo kweminwe kanye umsipha contracture - izimpawu zokuqala ukubhujiswa yezinzwa. Kamuva eba yoma nokukhubazeka kwemisipha. Isikhathi isilinganiso ukuphila iziguli oneminyaka ephakathi kuka 14 kuya ku 16.

isifo Late Verdniga-Hoffmann

isifo njalo is onomoya omnene. Njengomthetho, ingane eba ngempela ngokuvamile kuze umntwana eseneminyaka emibili. Kid ifunda ukuba ahlale, ame uhambe. Kuphela isikhathi abazali baqale ukuqaphela abanye ukuphambuka.

Okokuqala, ushintshe Gait ingane igula - lihambe kakhulu eguqa ngamadolo, kanengi uwela esonweni, ungababaleli ibhalansi. Njengoba zokugula ungaqaphela ezinye izinguquko kule skeleton, ezifana ukusonteka kwesifuba. Verdniga-Hoffman syndrome libhekene eliqhaqhazelayo eqinile, kwehle imisipha ithoni, ukunyamalala main ikhaliphe unconditioned.

Ezimweni eziningi iminyaka 10-12 ingane ilahlekelwa ngokuphelele ikhono ikhambo ezimele. Nokho, kulesi simo iziguli elisekhona 20, futhi ngezinye izikhathi kuze kube iminyaka engu-30.