Cerebromedullary khalad syndrome

Arnold-Chiari syndrome waa cillad-daran ee lagu dhasho ee madaxa maskaxda aadanaha. Its nuxurka been in faraq u dhaxeeya tirada fossa dambena waxay dhaladii, iyo kuwa kale ee ku yaala goobahan ee dhismayaasha maskaxda. Tani waxay keenaysaa in laga tegay qumanka xadgudub raacay cerebellum iyo stem maskaxda. Inta jeer ee cillado waa 4-8 qof 95-100 kun Man kasta..

Currently, pathogenesis cudurka aan la hubin. Ajnabiga, waxaa jira saddex arrimood pathogenic gacan ka disorder this:

1. lagu dhasho, neuropathy dhaxlo;
2. argagaxa dhaawac waslad-waslad-occipital iyo sol sagxada qayb sabab u ah dhaawacyada dhalashada;
3. dharbaaxo hydrodynamic CSF derbi ku dhacaya ee kanaalka laf-dhabarta, oo ku taalla bartamaha.

afar nooc oo cillado ayaa loo qoondeeyay ee 1891 Arnold Chiari, isticmaali soocidda this ilaa maantadan la joogo.

Arnold-Chiari nooca syndrome waa tirtirka dhakada fossa dambena iyo dhismayaasha ku xeeran ee kanaalka laf-aad uga yar tahay heerka Magnum kabsanayo. In nooca labaad ee cillad-waa-bax joogo caudal of oblongata medulla ah, meelaha ugu hooseeya ee dixirigoodu iyo ventricle IV ah, iyo ilaa xad inta badan loo horumariyo yiraahdo hydrocephalus. Arnold-Chiari syndrome waa nooc ka mid saddexaad waa mid aad u dhif ah, waayo, waxaa lagu gartaa oo dhan dhismayaasha aan dhab ahayn mowjadda maskaxda ee fossa dambena ka. Nooca afraad waxaa wakiil hypoplasia cerebellar. Noocyada afraad iyo saddexaad ee cillado kiisaska intooda badan, leheyn nolosha.

In 80% ee kiisaska, Arnold-Chiari syndrome waxaa lagu gartaa syringomyelia - cudurada of laf dhabarka lagu gartaa horumarinta fiix ee laf dhabarta, taas oo loo sameeyay by dambena tirtirka riixo fossa dhakada iyo gobolka afka ilmo-galeenka oo ah laf dhabarta. Joogitaanka fiix kuwaas keenaysaa in horumarka myelopathy horusocod ah.

Arnold-Chiari syndrome: Calaamadaha

Sawirka caafimaad ee cudurka waxaa lagu gartaa calaamadaha soo socda:

• xanuun qoorta iyo meelaha occipital, taas oo kordhinaysa marka hindhisada ama qufaca,
• hoos u heer-kulka iyo dareenka xanuunka, sidoo kale xoogga muruqa addimada sare;
• spasticiteten addinka,
• dawakhaad, diidmo, unsteadiness, xumeeyey madaxaaga jeestay, hoos toosan muuqaalka iyo tayada aragga.

Xaaladaha aadka u daran calaamadaha waafajiyaan kuwaas joojinta muddada-gaaban ah neefsashada (joogsiga), daciifisa of reflex pharyngeal iyo degdeg ah, dhaqdhaqaaq aan ikhtiyaarka lahayn ah ee indhaha.

Xaaladaha qaarkood, Arnold Chiari logu laga yaabaa in aanay wax calaamado ah oo si buuxda helay fursad lagu jiro nidaamka lagu ogaanayo. Cilad cudurka waxaa lagu fuliyaa iyadoo la isticmaalayo imaging MR iyo raajito loogu xisaabiyaa iyadoo dib u dhiska saddex dhinac-cabbir ah araxda afka ilmo-galeenka, iyo lafta occipital.

Arnold-Chiari syndrome: Daaweynta

daaweynta loo xilsaaray waxay ku xiran tahay bukaanka iyo darnaanta cudurka. Meelahaas waxaa astaamaha aasaasiga ah sida madax-xanuun iyo noocyada kale ee xanuun, dhakhtarkaaga ayaa laga yaabaa in dawada xanuunka iyo baaritaan si joogto ah. Bukaanjiifka qaar ayaa sheegay gargaarka marka la qaadanayo daawooyin anti-bararka in hortagi karaa ama xitaa dib u socod ah.

Inta badan, daaweynta, dhakhaatiirta door bidaan in ay ku qaataan aan caadi ahayn qalliin. Hawlgalka waa in la joojiyo horumarka ka mid ah isbeddelada qaabdhismeedka dhabarta iyo maskaxda iyo xasilinta calaamadaha. Marka fulinta vmeschatelstva guul qalliinka si weyn hoos u cadaadiska on laf-dhabarta iyo cerebellum, iyo sidoo kale soo kabsaday dheecaanka CSF ah. Waxaa jira farsamooyin kala duwan ee hawlaha in ay ku xiran tahay isagoo yiraahdo hydrocephalus. celceliska muddada qalliinka waa mid ka mid ah iyo badh ilaa laba saacadood, muddo kabashada qaadataa shan ilaa toddoba maalmood ah.