Cerebromedullary malformation syndrome

Arnold-Chiari syndrome ndi aakulu kobadwa nako anomaly mutu wa ubongo wa munthu. Kwenikweni umenewu chagona mu disparity pakati pa kukula kwa zapambuyo fossa a Chigaza, ndi anthu ena m'dera limeneli la nyumba ubongo. Zimenezi zimabweretsa kuchotsa anatsatira siudzalandilidwa ngathi lamulo tonsils cerebellum ndi ubongo tsinde. Pafupipafupi anomalies awa Nthawi 4-8 pa lililonse. Munthu 95-100 zikwi.

Panopa pathogenesis matenda ndi chosadziwika. Mwina, pali zinthu zitatu tizilombo zimathandiza kuti matendawa:

1. kobadwa nako, kumtundu neuropathy;
2. zoopsa choipa mphero-mphero-occipital ndi Grill gawo limbikitsa chifukwa cha zoopsa kubadwa;
3. CSF hydrodynamic nkhonya khoma la msana ngalande chingwe ili pakati.

amitundu inayi anomalies akhala allocated mu 1891 Arnold Chiari, ntchito gulu ili mpaka pano.

Arnold-Chiari syndrome mtundu ndi kufufutidwa zapambuyo fossa chigaza ndi nyumba ozungulira mu ngalande msana kwambiri m'munsi kuposa mlingo wa foramen magnum. Mu mtundu wachiwiri anomaly alipo caudal dislocation wa medulla oblongata, mbali m'munsi mwa mbozi ndi IV ventricle, ndipo nthawi zambiri kukhala hydrocephalus. Arnold-Chiari syndrome ndi mtundu wachitatu ndi osowa kwambiri, chifukwa amakhala ndi coarse kuchepetsa nyumba yonse ubongo wa zapambuyo fossa. Yachinayi ukufanizidwa ndi hypoplasia cerebellar. Wachinayi ndi wachitatu mitundu ya anomalies nthawi zambiri, atakwatirana ndi moyo.

80% milandu, syndrome Arnold-Chiari akapezeka ndi syringomyelia - kudwala a chingwe msana yodziwika ndi chitukuko cha cysts wa chingwe msana, amene anapanga ndi kufufutidwa zapambuyo fossa psinjika a Chigaza ndi dera khomo lachiberekero wa chingwe msana. Pamaso pa cysts izi zimabweretsa chitukuko cha mwapang'onopang'ono myelopathy.

Arnold-Chiari syndrome: Zizindikiro

Chithunzi matenda a matenda amakhala ndi zizindikiro zotsatirazi:

• kupweteka kwa khosi ndi malo occipital, amene kumawonjezera pamene sneezing kapena kutsokomola;
• kutsitsa kutentha ndi ululu tilinazo, komanso chapamwamba nthambi minofu mphamvu;
• nthambi spasticity;
• chizungulire, kukomoka, unsteadiness, kukula ndi kutembenuza mutu wako, utachepa acuity zithunzi ndi khalidwe la masomphenya.

Zikavuta kwambiri zizindikiro agwirizane ndi izi yochepa kutha kwa kupuma (apnea), ndi kufooka kwa pharyngeal reflex ndipo mofulumira, kayendedwe musanaganizire wa maso.

Nthawi zina, Arnold Chiari anomaly mwina zizindikiro zilizonse ndipo wapezeka kwathunthu mwangozi pa njira yodziwira. Matenda a matenda ikuchitika ntchito MR kulingalira ndi yopangidwa tomography ndi atatu azithunzi omwe tikunena kukhazikitsanso wa vertebrae khomo lachiberekero, ndi occipital fupa.

Arnold-Chiari syndrome: Chithandizo

Anatumizidwa chithandizo kumatengera pa mtima kwakukulu ndi matenda. Mu zizindikiro yaikulu monga mutu ndi zina ululu, dokotala wanu angakulangizeni ululu mankhwala ndi zonse cheke akuluakulu. Odwala ena lipoti mpumulo pamene mukumwa mankhwala odana ndi yotupa chimene chingalepheretse kapena sachedwa opareshoni.

Nthawi zambiri, chithandizo, madokotala amakonda kucheza kuvutika opaleshoni. ntchito ndi kusiya kupitirira za kusintha kwa msana ndi ubongo kapangidwe ndi olimba zizindikiro. Akamagwira bwino vmeschatelstva opaleshoni kwambiri amachepetsa kukakamiza chingwe msana ndi cerebellum, komanso akuchira ndi CSF outflow. Pali njira zosiyanasiyana za ntchito zimene Kukhala hydrocephalus. Kutalika pafupifupi opaleshoni ndi imodzi ndi theka maola awiri, nthawi kuchira amatenga masiku asanu ndi aŵiri.