Amyotrophy Spinal: omsakazo, izimpawu kanye ukuxilongwa

Okokuqala amyotrophy Spinal wayechazwa ekhulwini le-19 (ngo-1891 - Verdniga futhi 1893 - Hoffmann). Izimo zethu zihluke kakhulu isifo ezingavamile, okwenzeka nomuntu oyedwa ayizinkulungwane 100. Izifo iphathelene izifo zofuzo futhi esibonakala ngokuqina izilonda izicubu kwemisipha. Lezi zinqubo zihlobene izinqubo esiwohlozayo e izinzwa zezimpondo anterior we umgogodla nentambo motor nuclei stem ingqondo. Ngokuvamile, izimpawu zokuqala isifo avele esewusana noma ebuntwaneni. Izimpawu zincike uhlobo lwesifo futhi bangase balwisane njengoba hypotension, gipofleksii, abancela ukungasebenzi, ukuphefumula lapho uvuleka; ezimweni ezimbi kakhulu, lokhu zokugula has a umphumela ebulalayo. Ukuze lokwenza ukuxilongwa, kufanele ibhekane ukuhlolwa kwezakhi zofuzo.

amyotrophy Spinal: Izimpawu

Isithombe emitholampilo kuzoya ngohlobo isifo. Histology kwemisipha ejwayelekile ka neurogenic (okubangelwa ngenxa ukubhujiswa ezinzwa nezicubu) amyotrophy - ezingomakhelwane amaseli kwemisipha enempilo futhi onesifo. Mhlawumbe isici esiyinhloko sifo liyakhula ngobuthakathaka yoma imisipha zesivikelo sesifuba. Ngokuyinhloko kuthinta emikhawulweni proximal. With ukuthuthukiswa sifo inqubo amaqembu kwemisipha kancane kancane sokuthandana kanye nezinye. Ngokuvamile iziguli kungabonwa ukunyakazisa imisipha. izimpawu Isici zokugula kuyinto kyphoscoliosis, imisipha nokuphazamiseka kokuphefumula, ihlobene ukulimala imisipha intercostal. On radiographs ngokuvamile zembula thinning yokubekezela amathambo (humerus, kunawo wonke, njll).

Werdnig-Hoffmann Uhlobo lokuqala kubonakala zisencane ukuba ezinyangeni eziyisithupha. Phakathi nale nkathi, ingane hypotonia obhalisiwe kwemisipha, hyporeflexia, futhi fasciculations ulimi. Isici nobuntu lolu hlobo izifo nobunzima yokugwinya (dysphagia), oncelayo kanye emishinini yokuphefumula. Asphyxia e-95% amacala imbangela yokufa ezinganeni ezingaphansi kweminyaka engu-unyaka owodwa. Ngu iminyaka emine 100% ebulalayo.

Spinal amyotrophy Uhlobo lwesibili kuboniswa ezinyangeni 3-15 ubudala. Uma isifundo ingane umsipha ikhaliphe ezijulile bengekho, dysphagia kungenzeka. Izingane eziningi akakwazi ukukhasa uhambe, angaphansi kwamaphesenti 25 kungenziwa phansi. Ngokuvamile, isifo siphela ekufeni.

Spinal amyotrophy uhlobo lwesithathu, noma isifo, Kugelberg-Welander isifo, njengoba umthetho, bhalisiwe e yobudala kusukela ezingu-15 eminyakeni engu-19. Umtholampilo, njengasezulwini uhlobo mina, kodwa sifo sanda kancane kancane, ne eside ubude bempilo. Ubuthakathaka futhi kwesisindo iqala quadriceps imisipha femoral futhi flexor, ngokuhamba kwesikhathi kuvela izimpawu buphawuleka kakhulu futhi phezu imilenze. Ngemva nje kancane kwalokho, ngezandla ayaphazamiseka.

amyotrophy Spinal Eyesine kungenziwa ngofuzo, lesi sifo kubonakala abadala voraste kusukela eminyakeni engu-30 kuya ku-60. It libhekene ubuthakathaka, kwesisindo somzimba. Leli fomu differentsiirovat nzima kakhulu yesifo sokuwohloka kwezinzwa nemisipha.

amyotrophy Spinal Werdnig-Hoffmann kutholakala ukuthi uphethwe yini usebenzisa EMG (nerve conduction Isivinini kunqunywa, wenziwa isifundo imisipha, okuyinto innervated ngu nezinzwa cranial). Ukuze uqinisekise uphethwe kubalulekile ukuqhuba ukuhlolwa kwezakhi zofuzo, okuyinto ikhomba etiology yokwanda koshintsho e-95% amacala. izindlela engeziwe zokuxilonga zihlanganisa imisipha biopsies, ukutadisha umsebenzi enzyme (aldolase, creatine kinase).

oluthile lokwelapha lesi sifo alikho. Ukuze uvimbele ukuthuthukiswa contractures futhi ukugobeka komhlandla ebekiwe Physiotherapy futhi corset. amadivaysi Special wamathambo ukuvumela nesineke ukudla, bhala noma ukusebenza kwi-computer.