Spinal amyotrophy: etiology, symptoms a me kaʻike,

Ua ho'ākāka ke mua manawa Spinal amyotrophy i loko o ka 19th kenekulia (ma 1891 - Verdniga, a ma ka 1893 - Hoffmann). Muli o kona kulana pohihihi keia maʻi, 'ia i kekahi kanaka mai ka 100 tausani. Maʻi pili i ka aaiaoe illnesses, a ua wehewehe aku ma lesions o ka Muscle'aʻaʻa. Mau keʻano o ka hanaʻana i pili degenerative keʻano o ka hanaʻana i loko o neurons o ka anterior pepeiaohao o ka spinal kaula a me ka Hawaii nuclei o ka lolo kumu. I hookuu, o ka mua na hoailona o ka maʻi hōʻike iʻuʻuku mai ai kamaliʻi. Symptoms hilinaʻi ma luna o ka 'ano o ka maʻi, a e hoike i hypotension, gipofleksii, keiki omowaiu dysfunction, ha a me ka moni' ia; i loa na, keia pathology i ka ona e hoʻokūkū. I mea e hana i kaʻike,, oe e undergo aaiaoe ikea ai.

Spinal amyotrophy: Symptoms

Ka lapaʻau kiʻi, e hilinaʻi ma ka 'ano o ka maʻi. Histology o Muscle typical o neurogenic (kena ae la ma muli o ka luku ana o nā aʻalolo keena a me nā'aʻaʻa) amyotrophy - ke kaiāulu o ke ola a me ka maÿi Muscle keena. Paha ke kumu nui hiʻona o ka maʻi ua mahuahua nawaliwali, a me ka atrophy o ka Muscle olona. Kuhikuhi e loli ai proximalʻaoʻao. Me ka hooulu ana o ka maʻi i loko o ke kaʻina hana o ke kiʻekiʻe o ke komo pū o a me ka 'ē aʻe Muscle pūʻulu. Pinepine nā mea maʻi e ike hiki twitching nāʻiʻo. Ano hoailona o ka pathology mea kyphoscoliosis, Muscle contraction a me ka ha kāna hana, ka mea, ua pili me ka poho i ka intercostal nāʻiʻo. Ma radiographs pinepine hōʻike thinning o lōʻihi iwi (humerus, femur, etc.).

Werdnig-Hoffmann mua type ua hoikea mai oia, i ka i kakahiaka nui kona mau makahiki i ka eono mahina. I loko o kēia wā, ke keiki mea i kāinoaʻia muscular hypotonia, hyporeflexia, a me fasciculations 'ōlelo. A ano hiʻona o kēia 'ano o ka maʻi, he pilikia i ka moni' ia (dysphagia), keiki omowaiu, i pono me ka hanu. Asphyxia i ka 95% o ka hihia ma ka aoao o ka make no na keiki ma lalo o ka makahiki o ka hoʻokahi makahiki. Ka eha makahiki 100% ona.

Spinal amyotrophy lua o type ua hoike mai ia ma ka 3-15 malama o ka makahiki. Inā ke keiki study hohonu tendon reflexes ma kahi e, e hiki dysphagia. hiki ole ka hapanui o na keiki kolo a hele, emi o 25 pakeneka hiki ke noho. Pinepine, i ka maʻi welau a me ka make.

Spinal amyotrophy kolu o type, a me ka ahulau, Kugelberg-Welander maʻi, e like me ka rula, kakau ma ka makahiki mai ka 15 mahina a hiki i 19 makahiki. Ke? Aaieou, e like me ma ka 'ano aʻu, akā, i ka maʻi progresses mālie, me ka hou ke ola expectancy. Nawaliwali, a me ke kaupaonaʻana poho hoʻomaka me quadriceps femoral nāʻiʻo a me ka flexor, ma manawa o ka loa haʻi symptoms ikeia, a ma luna o nā wāwae. A iki ma hope, i ka lima i loli.

Spinal amyotrophy ha type hiki ke ili, ka maʻi hoike mai ai ia ia iho i loko o nā mākua voraste mai ka 30 a hiki i 60 makahiki. Ua wehewehe aku ma ka nawaliwali ka hoomaopopo ana, ke kaupaonaʻana poho Muscle. Kēia palapala ka loa paʻakikī differentsiirovat mai amyotrophic kīloi paʻewa sclerosis.

Spinal amyotrophy Werdnig-Hoffmann diagnosed ka hoʻohana 'ana EMG (nā aʻalolo conduction māmā holo me ka nānā', ua lawe mai i ka ao o nāʻiʻo, a i innervated e cranial nā aʻalolo). E hoʻokūpaʻa i kaʻike, e mea pono e hoʻomaka aaiaoe ikea ai, a e hōʻike ana i ka etiology o mutations i loko o 95% o ka hihia. Additional PalekanaEND_LINK ki ina hana like nā Muscle biopsies, ka study o ka ha awina o nā'ūhū (aldolase, creatine kinase).

I Kekahi Mau lapaʻau no keia maʻi hakuʻole. I mea e pale aku i ka ulu ana o contractures a me scoliosis hoakaka physiotherapy a me ka corset. Special orthopedic maalea ae i ka hoomanawanui i ka ai, kākau a hana ma ke kamepiula.