Kashin baya amyotrophy: etiology, cututtuka da kuma ganewar asali

A karo na farko kashin baya amyotrophy aka bayyana a cikin karni na 19th (a 1891 - Verdniga da kuma a 1893 - Hoffmann). Quite rare cuta, abin da ke faruwa da mutum daya daga dubu 100. Cuta da dangantaka da kwayoyin cututtuka da kuma halin da raunuka da tsoka nama. Wadannan matakai suna da alaka degenerative matakai a neurons na agara zankayen laka da kuma mota nuclei na kwakwalwa kara. Yawancin lokaci, na farko alamun cutar bayyana a jariri ko yara. Alamun dogara ne a kan irin cutar da zai iya bayyana a matsayin hypotension, gipofleksii, tsotsa tabarbarewa, numfashi da kuma hadiya. a mai tsanani lokuta, wannan Pathology yana da wani m sakamako. Domin yin ganewar asali, ya kamata ka sha kwayoyin gwaji.

Kashin baya amyotrophy: Alamun

The asibiti hoto zai dogara ne a kan irin cuta. Histology tsoka hali na neurogenic (sa saboda halakar jijiya Kwayoyin kuma tsokoki) amyotrophy - unguwa na lafiya da kuma xauke da cuta tsoka Kwayoyin. Zai yiwu babban alama da cutar yana karuwa wani rauni da atrophy na tsoka zaruruwa. Da farko rinjayar proximal extremities. Tare da ci gaba da cuta a cikin aiwatar da hankali shafa da kuma sauran tsoka kungiyoyin. Sau da yawa marasa lafiya za a iya gani twitching tsokoki. Halayyar ãyõyin Pathology ne kyphoscoliosis, tsoka ƙanƙancewa da numfashi cuta, shi ake dangantawa da lalacewar da intercostal tsokoki. A radiographs sau da yawa bayyanãwa thinning na dogon ƙasũsuwa (humerus, femur, da dai sauransu).

Werdnig-Hoffmann farko irin aka bayyana a farkon shekarun zuwa watanni shida. A wannan lokacin, da yaro ne rijista murdede hypotonia, hyporeflexia, kuma fasciculations harshe. A halayyar alama na da irin wannan cututtuka ne matsaloli a hadiya (dysphagia), tsotsa, kazalika da numfasawa. Asphyxia a 95% na lokuta dalilin mutuwar yara kasa da shekaru shekara guda. By shekaru hudu 100% m.

Kashin baya amyotrophy biyu irin aka nuna a cikin 3-15 watanni da shekaru. Idan yaro nazari mai zurfi agara reflexes ne ba ya nan, zai yiwu dysphagia. Mai yara ba zai iya ja jiki da kuma tafiya, kasa da kashi 25 za a iya zaunar da ku. Sau da yawa, cutar ƙare a mutuwa.

Kashin baya amyotrophy uku type, ko cuta, Kugelberg-Welander cuta, kamar yadda mai mulkin, rajista a shekaru daga 15 zuwa watanni 19 da shekaru. A asibitin, kamar yadda a cikin irin na, amma da cutar progresses sannu a hankali, tare da ƙara rayuwar jama'a. Rauninsa da kuma rashin nauyi asara fara da quadriceps femoral tsokoki da kuma flexor, a kan lokaci mafi furta bayyanar cututtuka bayyana, kuma a kan kafafu. A kadan daga baya, hannun shafa.

Kashin baya amyotrophy hudu irin za a iya gaji, cutar bayyana kanta a manya voraste daga 30 zuwa shekaru 60. Yana halin da rauni, nauyi asara tsoka. Wannan nau'i ne da wuya sosai differentsiirovat daga amyotrophic kaikaice sclerosis.

Kashin baya amyotrophy Werdnig-Hoffmann kamu amfani EMG (jijiya madugu gudu aka ƙaddara, yana da za'ayi a nazarin tsokoki, wanda aka innervated da girman kwanyar jijiyoyi). Don tabbatar da ganewar asali shi wajibi ne su gudanar da kwayoyin gwaji, wadda tayi da etiology na maye gurbi a cikin 95% na lokuta. Ƙarin bincike hanyoyin hada tsoka biopsies, da nazarin na ayyuka na enzymes (aldolase, creatine kinase).

Magani na musamman domin wannan cuta ba ya wanzu. Domin ya hana ci gaban contractures da scoliosis wajabta physiotherapy da corset. Special orthopedic na'urorin damar da mãsu haƙuri a ci, rubuta ko aiki a kan kwamfuta.