Syndrome malformation Cerebromedullary

Arnold-Chiari syndrome ko te Pākatokato congenital nui o te matenga o te roro tangata. takoto Ko tōna ngako i roto i te rerekëtanga i waenganui i te rahi o te posterior fossa o te angaanga, me etahi atu i roto i tenei rohe o te hanganga roro. arata'i tenei ki te mahuetanga aru tonsils hara roro tuarongo me te kakau roro. Ko te auau o enei kōhikohiko he 4-8 take ia nga 95-100 mano. Tangata.

I tēnei wā, he pahuhu noa te pathogenesis o te mate. Īhaka, i reira e toru ngā āhuatanga pathogenic whai wāhi ki tenei mate:

1. congenital, neuropathy tuku;
2. ngaukino whara poro-poro-kōpako me tunutunu rōnaki wahi rite ki te hua o te whētuki whanau;
3. pupuhi hydrodynamic CSF ki te taiepa o te awa ua, kei roto i te pokapū.

momo wha o kōhikohiko kua tohaina i roto i te 1891 Arnold Chiari, te whakamahi i tenei whakarōpū ki te ra nei.

Arnold-Chiari momo syndrome ko he nui raro atu i te taumata o te Magnum puta iti mukua angaanga posterior fossa me hanganga a tawhio noa i roto i te awa filosilivá. I roto i te momo tuarua o te Pākatokato he reira raruraru caudal o te oblongata medulla, nga wahi o raro rawa o te kutukutu, me te ana roro IV, a tino maha whakawhanake wairoro. Arnold-Chiari syndrome he he rawa onge he toru o te momo, hoki te āhuatanga reira e pokepoke, Paraire nga hanganga roro katoa o te fossa posterior. māngai te wha te momo e hypoplasia cerebellar. Nga momo wha, me te tuatoru o te kōhikohiko i roto i nuinga o te wā, hohotahi ki te ora.

I roto i te 80% o te wā, pāngia Arnold-Chiari syndrome te ki syringomyelia - māuiuitanga o te ua āhuatanga e te whanaketanga o cysts o te ua, e kua hanga e e mukua posterior fossa kōpeketanga o te angaanga, me te rohe waha o te ua. Ko te aroaro o enei cysts arata'i ki te whanaketanga o te myelopathy ahu whakamua.

Arnold-Chiari syndrome: Tohu

āhuatanga te pikitia haumanu o te mate e te tohu e whai ake nei:

• mamae i roto i te kaki, me ngā wāhi kōpako, e faarahi ka matihe maremare ranei;
• e tukupu te pāmahana, me te mamae tūtohutanga, me te runga te kaha wahi uaua;
• e alangá spasticity;
• whanoke, kaihapai, angatonu, hë e tahuri tou matenga, heke acuity ataata, me te kounga o te kite.

I roto i te wā tino kino hāngai tohu ki enei mutunga wā-poto o te kupukupu (apnea), te vaivai o te pūpū pharyngeal me tere, nekehanga tumu o te kanohi.

I roto i ētahi wā, Arnold Chiari kore ai Pākatokato i tetahi tohumate me te kitea rawa e tupono noa i roto i ngā tukanga tātaritanga. kawea tātaritanga o te mate i roto i te whakamahi i MR atahanga me tomography computed ki te hanga hou e toru-ahu o te pekehoe waha, me te wheua kōpako.

Arnold-Chiari syndrome: Treatment

Tei maimoatanga whakaritea i runga i te manawanui me te pakeke o te mate. I roto i enei tohu tuatahi rite ānini me ētahi atu momo o te mamae, kia tūtohu tō tākuta mamae rongoā, me te auau Taki-ups. korerotia ētahi tūroro tanga ka tango taero anti-inflammatory e taea te ārai ara whakaroa i tētahi mahi ranei.

Nuinga o ngā wā, maimoatanga, tākuta hiahia ki te noho abnormalities hāparapara. Ko te mahi, ko te ki te mutu te ahunga whakamua o te huringa o te tuaiwi me roro hanganga me te stabilization o tohumate. A, no te mau ana i roto i te vmeschatelstva hāparapara angitu tino heke te pēhanga ki runga i te ua, me te roro tuarongo, me te ora i te matamua CSF. He ngā tikanga o ngā mahi e whakawhirinaki i runga i he wairoro. Ko te roanga toharite o te pokanga ko tetahi me he hawhe ki e rua haora, e rima ki te whitu nga ra e te wā whakaora.