Spinal amyotrophy: etiology, zviratidzo uye kuongorora chirwere

Kekutanga Spinal amyotrophy akanga inorondedzerwa remakore rechi19 (muna 1891 - Verdniga uye muna 1893 - Hoffmann). Zvisinganyanyi kuitika chirwere, yokuvamba mumwe munhu kubva 100 zviuru. Disease chokuita magene zvirwere uye kunoratidzwa maronda ari tsandanyama tishu. azvaiitwa zvinofambidzana chinopedza pezvakaitwa mu Tutsinga tunofambisa mashoko utwu pamusoro Anterior nyanga musana uye injini nuclei uropi hunde. Kazhinji, zviratidzo chirwere yokutanga Akaonekwa mucheche kana vaduku. Zviratidzo kuvimba Chirwere uye vangapikisa sezvo hypotension, gipofleksii, yakayamwa dysfunction, kufema uye pakuparadza; muna Muzviitiko zvakaipisisa, zvokurwara ichi ane zvinouraya mugumisiro. Kuti chirwere, iwe unofanira kumbotambudzika magene yokuedzwa.

Spinal amyotrophy: Zviratidzo

The kunotoda mufananidzo achaenderana Chirwere. Histology kuti tsandanyama runowanzoitika neurogenic (kunokonzerwa nokuda kuparadzwa masero etsinga uye nyama) amyotrophy - pedyo utano uye nehosha tsandanyama masero. Zvichida huru chinoratidza maperembudzi kuwedzera simba uye atrophy yacho tsandanyama fibers. Zvikurukuru chinobata proximal migumo. With kukura hosha iri nedanho zvishoma zvaisanganisira uye mamwe mapoka tsandanyama. Kazhinji varwere vanogona kuonekwa twitching netsandanyama. Mamirire zviratidzo zvokurwara ndiko kyphoscoliosis, tsandanyama marwadzo uye zvinetso kufema, inobatanidzwa kuchikuvadza intercostal netsandanyama. On radiographs kazhinji zvinoratidza rive dete refu mapfupa (humerus, femur, etc.).

Werdnig-Hoffmann wokutanga mhando kunoratidzwa pazera duku kuti mwedzi mitanhatu. Panguva iyi, mwana vakanyoresa tsandanyama hypotonia, hyporeflexia, uye fasciculations mutauro. A maitiro kuchiitwa ichi mhando zvirwere zviri nematambudziko pakuparadza (dysphagia), yakayamwa, uyewo afemeruke. Asphyxia muna 95% nyaya chinokonzera rufu vana pasi pemakore gore rimwe. By makore mana 100% zvinouraya.

Spinal amyotrophy chechipiri mhando unoratidzwa 3-15 mwedzi zera. Kana mwana kudzidza zvakadzama mudzira hunenge hatisi, zvinogoneka dysphagia. Vana Most haigoni kukambaira uye kufamba, asingasviki 25 muzana kunogona pasi. Kazhinji, hosha kunoguma murufu.

Spinal amyotrophy mhando rechitatu, kana chirwere, Kugelberg-Welander chirwere, sezvo mutemo, vakanyorwa makore kubva kwemwedzi 15 kusvika makore 19. The kiriniki, sezvakunoitwa mhando ini, asi chirwere mberi zvishoma nezvishoma, pamwe hwakareba anotarisirwa. Kuneta uye uremu kunotanga quadriceps femoral tsandanyama uye flexor, pamusoro nguva vazhinji akazivisa zviratidzo kuoneka uye makumbo. Papera chinguva, maoko vanokanganiswa.

Spinal amyotrophy chechina mhando inogona nhaka, hosha kunooneka pacharo nedzevakuru voraste 30 kusvikira 60 makore. It hwokuratidza muutera, uremu tsandanyama. Iri fomu differentsiirovat zvakaoma chaizvo kubva chinonzi amyotrophic lateral sclerosis.

Spinal amyotrophy Werdnig-Hoffmann chirwere uchishandisa EMG (tsinga conduction velocity anoratidzwa, rinoitwa kudzidza tsandanyama, izvo innervated ne cranial tsinga). Kuzosimbisa chirwere zvakafanira kuti kuitisa majini yekuedzwa, iro rinotaura zviri etiology kuti kuno- muna 95% nyaya. Zvimwe chirwere nzira dzinosanganisira tsandanyama biopsies, kudzidzwa webasa makemikari (aldolase, creatine kinase).

Marapiro nokuti chirwere ichi haako. Kuitira kudzivirira pave contractures uye scoliosis ushandiswe physiotherapy uye corset. Special vachibatsira mano kubvumira murwere kudya, nyora kana kushanda pakombiyuta.