Msana amyotrophy: etiology, zizindikiro ndi matenda

Nthawi yoyamba msana amyotrophy limafotokonzera m'ma 19 (mu 1891 - Verdniga ndi 1893 - HOFFMANN). Ndithu osowa matenda, kunachitika munthu mmodzi 100 zikwi. Disease anafotokoza kuti matenda majini ndi amakhala ndi zotupa za minofu minofu. njira izi zokhudzana njira osachiritsika mu manyuroni nyanga zapambuyo ya msana ndi galimoto mtima pa tsinde ubongo. Kawirikawiri, zizindikiro woyamba wa matenda kuwonekera wakhanda kapena mwana. Zizindikiro zimadalira mtundu wa matenda ndi akhoza kusonyeza kuti hypotension, gipofleksii, woyamwa kukanika, kupuma ndipo Pomeza; Zikavuta, kudwala uyu ali ndi zotsatira amapha. Pofuna kuti matenda, inu ayenera kukayezetsa kukayezetsa chibadwa.

Msana amyotrophy: Zizindikiro

Chithunzi matenda kudzadalira mtundu wa matenda. Histology minofu ankaikira neurogenic (anachititsa chifukwa kuwonongedwa kwa maselo mitsempha ndipo zimakhala) amyotrophy - pafupi ndi maselo wathanzi ndi matenda minofu. Mwina mbali yaikulu ya matenda zikuchulukirachulukira kufooka ndi manja a minofu ulusi. Makamaka umakhudza m'makona proximal. Ndi chitukuko cha matenda mu ndondomeko ya magulu pang'onopang'ono mtsikana ndi zina minofu. Nthawi zambiri odwala Tingaone kukokana minofu. Zizindikiro khalidwe la kudwala ndi kyphoscoliosis, minofu chidule ndi kupuma matenda, amagwirizanitsidwa ndi kuwonongeka kwa minofu intercostal. Pa radiographs zambiri awulule kupatulira wa yaitali mafupa (humerus, femur, etc.).

Werdnig-HOFFMANN yoyamba akuwonekera ali wamng'ono miyezi isanu ndi umodzi. Nthawi imeneyi, mwanayo olembetsedwa akulu hypotonia, hyporeflexia, ndi chinenero fasciculations. Mbali khalidwe la mtundu wa matenda ndi mavuto Pomeza (dysphagia), woyamwa, komanso kupuma. Asphyxia mu 95% milandu chifukwa cha imfa kwa ana osakwana zaka chaka chimodzi. Ndi zaka zinayi 100% amapha.

Msana amyotrophy mtundu chachiwiri chimene chikuoneka pa miyezi 3-15 zakubadwa. Ngati phunziro mwana kwambiri tendon bongo ndi kwina, nkotheka dysphagia. Ana ambiri sangathe kukwawa ndi kuyenda peresenti zosakwana 25 akhoza kukhala pansi. Nthawi zambiri, matenda itha imfa.

Msana amyotrophy lachitatu mtundu, kapena matenda, matenda Kugelberg-Welander, monga ulamuliro, mayina zaka miyezi 15 mpaka zaka 19. Chipatala, monga mwa mtundu Ine, koma matendawa pang'onopang'ono, ndi moyo wautali zambiri. Zofooka ndi kuwonda akuyamba ndi quadriceps minofu femoral ndi flexor, pa nthawi zizindikiro kwambiri kutchulidwa kuoneka ndi miyendo. Patapita kanthawi, manja akhudzidwa.

Msana amyotrophy yachinayi akhoza wobadwa nawo, matenda kumaonekera akulu voraste kuyambira azaka 30 mpaka 60. Iwo yodziwika ndi kufooka, kuwonda minofu. fomu iyi ndi zovuta differentsiirovat ku amyotrophic lateral sclerosis.

Msana amyotrophy Werdnig-HOFFMANN anapezeka ntchito EMG (mitsempha conduction mathamangidwe amaonekera ikuchitika kuphunzira minofu, amene innervated ndi misempha cranial). Kutsimikiza matenda m'pofunika kuchita kuyezetsa chibadwa, amene ndikuzindikiritsa etiology cha masinthidwe 95% ya milandu. njira zina matenda monga biopsies minofu, kuphunzira ntchito za michere (aldolase, creatine kinase).

mankhwala enieni matenda kulibe. Pofuna kupewa chitukuko cha contractures ndi kupindika msana Kwalamulidwa physiotherapy ndi corset. Special zipangizo mafupa kulola mtima kudya, kulemba kapena ntchito pa kompyuta.