Amyotrophy filosilivá: etiology, tohu me te tātaritanga

whakaahuatia te wā tuatahi amyotrophy ua i i roto i te rau tau 19 (i roto i te 1891 - Verdniga me i roto i 1893 - Hoffmann). Tino mate onge, puta tetahi tangata i 100 mano. Pā ana mate ki mate iranga, me te e āhuatanga e pāpaka o te kiko uaua. E pā ana ēnei tukanga tukanga degenerative i roto i neurons o nga haona he mārōrō, o te aho, me te nekeneke karihi filosilivá o te kakau roro. Ko te tikanga, puta nga tohu tuatahi o te mate i roto i te aruaruraa kōhungahunga ranei. Symptoms whakawhirinaki i runga i te momo o te mate, me te kia whakaatu rite hypotension, gipofleksii, ngote pōrearea, te kupukupu me te horomia; i roto i ngā take kino, tenei māuiuitanga he putanga mate. I roto i te tikanga ki te hanga i te tātaritanga, kia amohia e koe whakamātau ira.

amyotrophy tuaiwi: Tohu

Ka whakawhirinaki te pikitia haumanu i runga i te momo o te mate. Histology o te uaua angamaheni o neurogenic (meinga e tika ana ki te whakangaromanga o ngā pūtau io me te kopa) amyotrophy - te tata o ngā pūtau uaua hauora, me te mate. Pea kei te piki haere te āhuatanga matua o te mate ngoikore, me te atrophy o te uaua muka. Matua pā pito proximal. Ki te whanaketanga o te mate i roto i te tukanga o ngā rōpū uaua āta whai wāhi, me te tahi atu. Pinepine e taea te kite i ngā tūroro aitua, uaua. tohu āhuatanga o te māuiuitanga he kyphoscoliosis, uaua hukihuki, me te kupukupu tonu disorders, kua whai pānga te reira ki kino ki te uaua intercostal. I radiographs maha whakakitea thinning o roa wheua (peke, pūkaka, me ētahi atu).

whakakitea Werdnig-Hoffmann momo tuatahi te i te wawe tau ki te ono marama. I roto i tenei wā, te tamaiti he hypotonia rēhita uaua, hyporeflexia, me fasciculations reo. He āhuatanga āhuatanga o tenei momo o mate he fifi i roto i horomia (dysphagia), ngote, me te respiration. Asphyxia i 95% o ngā take te take o te mate mō ngā tamariki i raro i te tau o te tau kotahi. Na roto i te wha tau e 100% mate.

whakaaturia filosilivá amyotrophy momo tuarua i roto i te 3-15 marama o te tau. Ki te he mea motu te ako tamaiti reflexes iohere hohonu, dysphagia taea. e kore e taea e te nuinga o ngā tamariki ngōki ka haere, ka taea e iti iho i te 25 ōrau e noho. E mea pinepine, mutu te mate i roto i te mate.

Filosilivá amyotrophy momo tuatoru, ranei mate, mate Kugelberg-Welander, rite ki te tikanga, rēhita i roto i te tau i te 15 marama ki te 19 tau. Te whare hauora, pera i te momo ahau, engari ko te mate kauneke āta, me roa tümanako ora. Ngoikore, me te mate taimaha haamata ki whā uaua femoral me flexor, i runga i te wā puta nga tohu tino korerotia me ki runga ki nga waewae. A iti i muri mai, kua pāngia te ringa.

amyotrophy filosilivá taea te riro momo tuawha, faaite te mate ano i roto i ngā pakeke voraste i 30 ki te 60 tau. āhuatanga i te reira na roto i te ngoikore, mate taimaha uaua. Tenei puka Ko differentsiirovat rawa uaua i sclerosis lateral Amyotrophic.

amyotrophy filosilivá Werdnig-Hoffmann pāngia te whakamahi i EMG (takoto karere āriponga tere te, kei te kawea i roto i te ako o uaua, e kua innervated e raru cranial). Hei whakaū i te tātaritanga he mea e tika ana ki te whakahaere whakamātau ira, e tāutu ana i te etiology o ngā irakētanga i roto i 95% o te wā. Atu tikanga tātaritanga ngā biopsies uaua, te ako o te mahi o whākōkī (aldolase, creatine kinase).

e kore e maimoatanga motuhake mō tenei mate te tīariari. I roto i te tikanga ki te ārai i te whanaketanga o contractures me scoliosis whakaritea i tua me te koere. pūrere orthopedic Special tukua te manawanui ki te kai, tuhituhi mahi i runga i te rorohiko ranei.