Amyotrophy spinal: umyalezo, iimpawu kunye isifo

Ixesha lokuqala amyotrophy Spinal kuchazwa ngenkulungwane ye-19 (ngo-1891 - Verdniga kwaye ngo-1893 - Hoffmann). Lidla isifo ezinqabileyo, elivela umntu omnye ukususela 100 lamawaka. Izifo inxulumene izigulo yemfuza luphawulwa izitshanguba ze ijwabu kwezihlunu. Ezi iinkqubo ezinxulumene iinkqubo sisiba mandundu xa luvo ezimpondweni Icipande i-spinal intambo kunye motor eziba izikhondo ebuchotsheni. Ngokuqhelekileyo, imiqondiso yokuqala zezifo kuvela uzaze okanye ebuntwaneni. Iimpawu zixhomekeka uhlobo lwesifo kwaye ibonakale njengoba hypotension, gipofleksii, usana kwekhono, ukuphefumla nokuginya; kwiimeko ezinzima, le ngezifo sele esibulalayo. Ukuze wenze isifo, kufuneka benze uvavanyo yemfuza.

amyotrophy spinal: Iimpawu

Lo mfanekiso zizehlo iya kuxhomekeka kuhlobo sifo. Histology zihlunu obabuthandwa neurogenic (yabangela ngenxa kwintshabalalo iiseli zemithambo-luvo kunye emzimbeni) amyotrophy - elumelwaneni lwaloo iiseli kwezihlunu esempilweni yaye egula. Mhlawumbi iphambili esi sifo liyenyuka ubuthathaka kunye yoma kwemisipha imicu. Ubukhulu becala kuchaphazela yesiqephu proximal. Kunye nophuhliso sifo kwinkqubo amaqela kwezihlunu ngokuthe ngcembe inxaxheba kunye nezinye. Kaninzi izigulane ingabonwa twitching izihlunu. Iimpawu uphawu ngezifo na kyphoscoliosis, imisipha kunye nengxaki ukuphefumla, inxulunyaniswa umonakalo kwizihlunu intercostal. On radiographs badla zityhila thinning of elide amathambo (ihumerus, yifemur, njalo njalo).

Werdnig-Hoffmann Uhlobo lokuqala lubonakala esemncinane ukuya kwiinyanga ezintandathu. Ngeli xesha, umntwana hypotonia ebhalisiweyo dystrophy, hyporeflexia, kunye fasciculations ulwimi. Eyona uphawu olu hlobo izifo ubunzima yokuginya (dysphagia), na usana lwakhe, ngokunjalo nokuphefumla. Zizalwa ngo-95% yamatyala unobangela wokufa kwabantwana abangaphantsi kweminyaka konyaka omnye. Ngu kweminyaka emine esibulalayo 100%.

Spinal amyotrophy Uhlobo lwesibini kubonisiwe kwiinyanga 3-15 ubudala. Ukuba ngomntwana ikhaliphe imicu nzulu abangekhoyo, dysphagia kunokwenzeka. Uninzi lwabantwana akakwazi lonwabu ndihambe, ngaphantsi kwama-25 ekhulwini kunokuba phantsi. Ngokufuthi, esi sifo kuphelela ekufeni.

amyotrophy Uhlobo spinal wesithathu, okanye isifo, isifo Kugelberg-Welander, njengoko umthetho, ebhaliswe ubudala ukususela kwiinyanga-15 ukuya kwiminyaka-19. Ikliniki, njengokuba uhlobo mna, kodwa isifo siqhubela kancinci, amade sokuphila. Yokuswela amandla, ukwehla kobunzima luqala quadriceps izihlunu femoral kunye flexor, ngokuhamba kwexesha kuvela iimpawu ezininzi lwaziwayo apha emilenzeni. Kamva, izandla ayachaphazeleka.

amyotrophy spinal Uhlobo lwesine unako ilifa, esi sifo esizibonakalisa abadala voraste ukusuka kwiminyaka engama-30 ukuya kuma-60. It luphawulwa ubuthathaka, ukwehla kobunzima kwezihlunu. Le fomu differentsiirovat nzima kakhulu ukusuka lateral sclerosis.

amyotrophy spinal Werdnig-Hoffmann kwafunyaniswa ukuba usebenzisa EMG (nemithambo Uhanjiso Velocity imiselwa, ikhutshwa phandle isifundo izihlunu, leyo innervated yi luvo cranial). Ukuze uqinisekise uxilongo oko kuyimfuneko ukuqhuba uvavanyo yemfuza, nto leyo ichaza ukuba umyalezo kohlo- ngo-95% yamatyala. iindlela ezongezelelweyo diagnostic ziquka biopsies kwezihlunu, isifundo yomsebenzi enqanda (aldolase, creatine kinase).

unyango oluthile esi sifo ayikho. Ukuze kuthintelwe uphuhliso contractures kunye zokufota emiselweyo physiotherapy kunye corset. izixhobo ezikhethekileyo wamathambo ukuba isigulane ukuba adle, ukubhala okanye ukusebenza kwi computer.