Werdnig-Hoffmann: siffar da kuma babban bayyanar cututtuka

Werdnig-Hoffmann - musamman tsanani Pathology wanda yana tare da amyotrophy, hankali halakar da babban jijiya zaruruwa na laka, kuma, saboda haka, tsoka atrophy. Nan da nan shi ne ya kamata a lura da cewa wannan shi ne wani gaji kwayoyin cuta, wadda aka ruwaito a wani autosomal recessive hanya.

Mene ne Werdnig-Hoffmann?

Yana da wani neurological cutar tare da sauka a hankali halakar da asali Tsarin daga cikin juyayi tsarin innervating. Alal misali, sau da yawa akwai demyelination na agara tushen da laka. Bugu da kari, cutar rinjayar wasu girman kwanyar jijiyoyi.

Hakika, da lalacewar da jijiya zaruruwa rinjayar da jihar na tsokoki. Duk da haka, domin wannan cutar halin da ake kira katako tsoka hasãra, a cikin abin da ɓangare na tsoka rike da ikon zuwa mu'ãmalar, yayin da raba "daure" atrophy.

Kashin baya atrophy Verdniga-Hoffmann bayyana a cikin shimfiɗar jariri. Yau ne zuwa kashi uku main siffofin da cutar.

Nakasar cuta Verdniga-Hoffmann da kuma ta bayyanar cututtuka

Yawanci, da farko bayyanar cututtuka da wannan nau'i na cutar su ne riga m, a farko 'yan kwanaki bayan haihuwa. The yaro gabatar kasãla paresis na extremities. Kukan rashin lafiya yara ne mai rauni, kuma kawai audible, a Bugu da kari, suna da ikon tsari ne karye.

Kamar yadda yaro ke tsiro, za ka iya lura da fada bayan a zahiri ci gaba. Marasa lafiya jariran ba ci gaba da kai, za su iya ba zauna kuma kada ka tsaya. Kawai a rare lokuta, da jariri ne iya rike jiki a karkata zuwa ga gaskiya matsayi, amma wannan ikon vuya fairly da sauri kamar yadda halakar jijiya zaruruwa.

Bugu da kari, akwai wasu munanan a kwarangwal ci gaba, musamman hip dysplasia, scoliosis, da samuwar mazurari kirji, kwancen kafa, hydrocephalus.

A lokacin m cuta, da kuma degeneration na jijiya endings aka ci gaba cikin sauri. Atrophy za a iya bayyana ba kawai da kwarangwal tsokoki, amma kuma zaruruwa na ciki gabobin. Sau da yawa shafi diaphragm, wadda take kaiwa zuwa numfashi gazawar. Abin baƙin ciki, yara da irin wannan cuta rayuwa (a kan talakawan) har zuwa shekara tara.

Farkon Verdniga-Hoffmann cuta

Babban bayyanar cututtuka da cutar bayyana kansu a karo na biyu da rabi na rayuwa. Na farko 'yan watanni na jiki ci gaban da yaro ya wuce quite al'ada - yaro san rike da kai da kuma zama, wani lokacin ma iya zama har unaided. Amma duk da waɗannan dabarun an rasa bayan da kunnawa da cutar. Af, sau da yawa tsokani kamuwa ciwo.

Tsãwa na yatsunsu da agara contracture - da farko alamun da lalata jijiya zaruruwa. Daga baya tasowa atrophy kuma inna daga cikin tsokoki. A talakawan tsawon rai da marasa lafiya ne tsakanin 14 zuwa 16 da shekaru.

Late cuta Verdniga-Hoffmann

Irin wannan cuta ne milder. Matsayin mai mulkin, da yaro tasowa quite kullum har da shekaru biyu. The yaro san su zauna, tsaya da tafiya. Kawai tare da lokaci iyaye su fara lura da wasu karkacewa.

Da farko, canja Gait wata rashin lafiya yaron - ya ke, yawa lankwasawa da gwiwoyi, sau da dama, ba su rike da sikeli. Kamar yadda Pathology iya lura da wasu canje-canje a cikin kwarangwal, kamar nakasawa da kirji. Verdniga-Hoffman ciwo halin da karfi tremor, rage tsoka sautin, da bacewar babban unconditioned reflexes.

A mafi yawan lokuta 10-12 shekaru na yaro gaba daya hasarar da ikon da m tafiya. Duk da haka, a cikin wannan yanayin da marasa lafiya tsira zuwa 20, da kuma wani lokacin har zuwa shekaru 30.