Werdnig-Hoffmann: i ka palapala, a me ka papa kuhikuhiE symptoms

Werdnig-Hoffmann - loa mea pathology i ua pu ma ka amyotrophy, gradual ka make ana o ke kumu nui nā aʻalolo olona, o ka spinal kaula, a, nolaila, Muscle atrophy. Koke ia mea kumukuai 'ana i kumumanaʻo i keia mea he ili aaiaoe maʻi, i ua pū i hiki i ka autosomal recessive ano.

He aha ka Werdnig-Hoffmann?

He He neurological maʻi pu ma o ka holo loa ana o ke kumu o nā hale o ka'emi ko kahua innervating. No ka laʻana, pinepine he mea demyelination o ka anterior aa o ka spinal kaula. Eia hou, i ka maʻi e loli ai kekahi cranial nā aʻalolo.

O ka holo ana, no ka pōʻino i ka nā aʻalolo olona, e loli ai i ka moku'āina o na nāʻiʻo. Eia naʻe, no ka mea, i kēia maʻi ua wehewehe aku ma ka ai-kapaia mea nana i ulana Muscle poho, ma i hapa o ka Muscle i hoopaa mai ai i ka hiki i ka palapala, oiai ke kaawale "pua" atrophy.

Spinal atrophy Verdniga-Hoffmann hoikea mai oia, i loko o kamaliʻi. I keia la ua maheleia i ekolu papa kuhikuhi ano o ka maʻi.

Congenital maʻi Verdniga-Hoffmann a me kona symptoms

Nō kāu, o ka mua symptoms o keia palapala o ka maʻi i, ua kekahi 'i loko o ka mua mau lā ma hope o ka hanau ana. Ke keiki hoike sluggish paresis o nāʻaoʻao. Na hea ana o na keiki maʻi, ua nawaliwali, a māhunehune audible, ma ka hou, ka mea, i ka mana kaʻina hana ka mea i haki.

E like me kāu keiki ulu, oe ke hai i ke lag ma physical ulu. Maʻi keiki e, mai malama i ke poo, hiki aole noho, aole hoi ku. Wale i loko o pohihihi keia hihia, ke kamaiki mea hiki ke hoopaa i ke kino i loko o ka pololei kulana, akā, i kēia hiki nalo 'ano koke like me ka make ana o ke aʻalolo olona.

Eia hou, aia nō kekahi abnormalities ma skeletal kūpono 'ana, pakahi aku la ia ke ki kala dysplasia, scoliosis, i ke kahua o ka funnel umauma, clubfoot, hydrocephalus.

Iloko o ka malignant maʻi, a me ka degeneration o nā aʻalolo a hāʻule pahū ua holo pono hikiwawe loa. Atrophy hiki ke ho'ākāka 'ia ma ole wale ka skeletal nāʻiʻo, akā, i ka olona, o ka mea na nā loko. Pinepine loli ka diaphragm, a hiki aku i ka ÷ naehana hanu ole. Eia naʻe, na keiki me ka like maʻi ola (ma ka awelika) mai a hiki i ka makahiki o ka eiwa.

I kakahiaka nui Verdniga-Hoffmann maʻi

I ka papa kuhikuhiE symptoms o ka maʻi hoike mai ai ia lakou iho i loko o ka lua o ka hapalua o ke ola. Ka mua mau mahina o ka physical? Acaeoey o ke keiki hele nō maʻamau - i ke kao keiki e ao hou e hoopaa i ke poo a me ka noho, i kekahi manawa, a hiki ke noho i unaided. Akā, ua lilo ma hope o ka ho'ā hope o ka maʻi i kēia mau mākau. Ma ke ala, pinepine ka hoʻonāukiukiʻana maʻiʻaʻai maʻi pale.

Tremor o nā manamana lima a me ka tendon contracture - ka mua hoailona o ka make ana o ke aʻalolo olona. Hope e hoʻomōhala ana i atrophy a me lōlō o na nāʻiʻo. Ka awelika lōʻihi like 'ole o ke ola o nā mea maʻi mea ma waena o 14 a hiki i 16 makahiki.

Late maʻi Verdniga-Hoffmann

Oia mai ka milder. E like me ka rula, i ke keiki e hoʻomōhala ana iʻano maʻamau a ka makahiki o elua. Ke kao keiki e ao hou i ka noho, ku a hele. Wale me ka manawa makua hoʻomaka e hai i kekahi haiahū kūmau.

Mua, e hoʻololi i ka gait o ka maʻi keiki - hele, nui anuenue na kuli, hina pinepine ia, mai e hoopaa i ke koena. E like me ka pathology i hai i kekahi loli ma ka iwi, e like me deformation o ka pahu. Verdniga-Hoffman maʻi pale ua wehewehe aku ma ka tremor ikaika, ua hoʻemi Muscle leo, i ka nalowaleʻana o ka papa kuhikuhiE unconditioned reflexes.

Ma ka hapanui o na hihia 10-12 makahiki o ke keiki loa haalele i ka hiki ana o kuokoa Hotele. Naʻe, i loko o keia hihia na nā mea maʻi ola mahope aku o ke 20, a me kekahi manawa, i ka 30 makahiki.