Lesapo la mokokotlo amyotrophy: etiology, matšoao le phumano

Ka lekhetlo la pele lesapo la mokokotlo amyotrophy ne a hlalosoa a lekholo la bo19 la lilemo (ka 1891 - Verdniga le ka mobu 1893 ete - Hoffmann). Haholo ka seoelo lefu, etsahalang motho a le mong ho tloha 100 tse sekete. Mafu amana mafu a liphatsa tsa lefutso le e tšoauoa ka diso tsa mesifa dinama tse nyenyane tse. dithulaganyo tsena li amanang le dithulaganyo holofatsang ka methapo ea kutlo tsa manaka anterior ea lesapo la mokokotlo thapo le dilenaneo nuclei ba bakoang boko. Hangata, lipontšo pele la lefu hlaha ka boseeng kapa bongoaneng. Matšoao a itšetleha ka mofuta oa boloetse le ka 'na ka bontša ka hypotension, gipofleksii, anyang ho se sebetse, ho hema le ho engole; maemong a matla, bolwetse ena e na le sephetho le bolaeang. E le ho etsa hlahloba, o lokela ho feta liteko liphatsa tsa lefutso.

Lesapo la mokokotlo amyotrophy: Matšoao

The setshwantsho ea tleleniki e tla itšetleha ka mofuta oa lefu. Sobotta a mesifa tloaelehileng neurogenic (bakileng ka lebaka la ho timetsoa ha lisele tsa methapo ea kutlo 'me lisele tse) amyotrophy - motseng lisele tse phetseng hantle' me a kula mesifa. Mohlomong tšobotsi e ka sehloohong ea lefu lena le ho eketseha bofokoli le felloa ke matla a mesifa likhoele. Haholo-holo e ama lipheletsong proximal. Le ntshetsopele ya lefu lena le ka tshebetso ya ho lihlopha butle-butle e amehang le tse ling tse mesifa. Boo hangata bakuli ba ka bonoa twitching mesifa. matšoao bath ya bolwetse e kyphoscoliosis, mesifa e honyele le ho hema mafu a, e amanang le ho senya mesifa intercostal. Ka radiographs hangata e senola thinning ea nako e telele masapo (humerus, femur, joalo-joalo).

Werdnig-Hoffmann mofuta pele e bontšoa a sa le monyenyane ho likhoeli tse tšeletseng. Nakong ena, ngoana a ngodisitsweng la mesifa hypotonia, hyporeflexia, 'me puo fasciculations. A tsobotsi bath ea mofuta ona wa mafu ke mathata a engole (dysphagia), anyang, hammoho le respiration. Asphyxia ka 95% ea linyeoe sesosa sa lefu bakeng sa bana ba ka tlaase ho lilemo tse selemo se le seng. Ke lilemo tse 'nè tse 100% bolaeang.

Lesapo la mokokotlo amyotrophy mofuta bobeli e bontša ka likhoeli tse 3-15 ba lilemo. Ha ho ithuta ngoana tebileng tendon reflexes ke le sieo, khoneha dysphagia. bana ba bangata ba sitoa ho khasa 'me u tsamaee, ba etsang liphesente tse ka tlase ho 25 ka lutse. Hangata, lefu lena le e qetella ka lefu.

Lesapo la mokokotlo amyotrophy boraro mofuta, kapa maloetse, lefu Kugelberg-Welander, e le busa, ngolisitsoeng tsa lilemo tse likhoeli tse 15 ho ea ho lilemo tse 19. Tleliniki, joalokaha e etsahala mofuta na, empa ke lefu le sa tsoela pele butle-butle, ka nako e telele ba phela. Bofokoli le boima ba 'mele e qala ka quadriceps mesifa femoral le flexor, ka mor'a nako matšoao a ho fetisisa bitsa hlaha le ka maoto a le. Nakoana ka mor'a matsoho a ameha.

Lesapo la mokokotlo amyotrophy mofuta bone ka seo re se futsitseng, lefu lena le bontša ka boeona e ka batho ba baholo voraste tloha ka ba lilemo tse 30 ho tse 60. E tšoauoa ke bofokoli, boima ba 'mele mesifa. foromo ena e thata haholo differentsiirovat ho tloha lefu la 'amyotrophic lateral sclerosis.

Lesapo la mokokotlo amyotrophy Werdnig-Hoffmann fumanoa sebelisa baleg emg (methapo ea kutlo conduction lebelo ikemiselitse, e etsoa ka ho ithuta mesifa, tse innervated ke methapo cranial). Ho netefatsa tepelletse maikutlong le hore ke ho hlokahala hore ho tsamaisa liteko liphatsa tsa lefutso, e leng e khetholla le etiology ba liphetoho liphatseng tsa lefutso ka 95% ea linyeoe. mekhoa eketsehileng tekotshupo kenyeletsa biopsies mesifa, ho ithuta mosebetsi oa enzyme (aldolase, creatine kinase).

kalafo e itseng bakeng sa lefu lena ha e yo. E le hore ho thibela ntshetsopele ya contractures le scoliosis balletsweng ea physiotherapy le corset. Special disebediswsa masapo lumella mokuli ho ja, ngola kapa sebetsa ka k'homphieutha.