Alexander cuta. Form. Ganewar asali. Outlook

Alexander cuta - shi ne mai matukar rare neurological Pathology wanda shi ne m, a yanayi. A dalilan da wannan pathological yanayin da ya ba tukuna aka gano ba, amma karfi matsayi na maye gurbi ka'idar riqe GFAP gene. A gene alhakin shigar glial fibrillary acidic gina jiki.

Alexander cuta auku sporadically, akwai bayanai game da m iyalinmu lokuta da cutar. Gaji cuta ne autosomal rinjaye gado irin. A lokacin, masana kimiyya saki uku irin wannan cuta: adult, matasa da kuma yaron.

A farko manifestations infantile nau'i na cutar fara kewaye da lokaci yaro ne 6 watanni. Babban bayyanar cututtuka iya kira pathological girma na kai, kazalika da neurological cuta. Akwai jinkiri a psychomotor da kuma ta jiki ci gaba. Akwai wasu extrapyramidal cuta kamar dystonia da horeartroz. Iya samar da spastic tetraparesis. Bayan duk wannan, da yaro da aka ciwon wahala a hadiya. Lokaci-lokaci mai haƙuri iya lura nystagmus, kazalika hanyar spontaneously motsi da eyeballs. Wani lokaci haƙuri yana barci apnea. A kan talakawan, marasa lafiya rayuwa 2-3 shekaru bayan na farko da bayyanar cututtuka bayyananne. Cututtuka na juyayi tsarin a yara ne mai hadarin gaske ga fledgling jiki.

Alexander cuta, wanda yake malala a cikin nau'i na matasa, suka fara samar a lokacin daga 4 zuwa shekaru 14. Retrospective analysis bayyana cewa primary cututtuka kadan a baya: kafin shekaru biyu yara a baya a psychomotor ci gaba, sau da yawa da epilepsy, kuma sannu a hankali kafa tetraparesis yiwu pseudobulbar cuta, m apnea aukuwa. Mafi yawa daga cikin marasa lafiya saukar macrocephaly, amma ba za a iya sa a layi tare da halayyar siffofin da wannan cuta a cikin infantile lokaci. Sau da yawa, marasa lafiya fama da unexplained bouts na amai, musamman a cikin safe. A hankali, shi kiwon wata karuwa da pyramidal cuta kamar ataxia, kwakwalwa seizures. Shi ne ya kamata a lura da cewa ilimi aiki ba ya canzawa. MRI Alexander cuta (hoto da aka nuna a kasa) da aka bayyana hankula canje-canje. Rayuwa Hasashen - 7-9 shekaru daga farko na babban bayyanar cututtuka.

Very bambancin a asibiti manifestations adult nau'i na cutar. Daga na biyu har bakwai shekaru goma na rayuwa bikin farko na bayyanar cututtuka. A hankali a marasa lafiya nuna alamun cerebellar da corticospinal yankuna. Kadan fiye, shi za a iya gani nystagmus da hanawa da fahimi aiki.

Babban Hanyar ganewar asali da wannan cuta tsari ne MRI, CT, kuma DNA analysis. Magani na musamman domin Alexander cuta yana ba. Jiyya ne symptomatic. Hangen nesa ne talakawa da kuma dogara a kan yadda sauri da manifesto Pathology. A jima da wannan zai faru, da sauri da mutuwa.