Cerebromedullary malformation syndrome

Arnold-Chiari syndrome bụ a oké congenital anomaly isi nke ụbụrụ mmadụ. Ya kachasi mkpa idu ke kara n'etiti size of posterior fossa nke okpokoro isi, na ndị ọzọ na mpaghara ebe a nke ụbụrụ owuwu. Nke a na-eduga ná nhapụ soro ịda iwu tonsils cerebellum na ụbụrụ azuokokoosisi. The ugboro ole nke ndị a anomalies bụ 4-8 ikpe kwa bụla 95-100 puku. Man.

Ugbu a, ndị pathogenesis nke ọrịa bụ na-ejighị n'aka. Presumably, e nwere atọ pathogenic so na-eme ka nsogbu a:

1. congenital, butere n'aka neuropathy;
2. traumatic mmerụ nkpirikpi-nkpirikpi-occipital na imi òkè ramp ka a N'ihi nke a ọmụmụ trauma;
3. CSF hydrodynamic igbu megide mgbidi nke ọgidigi azụ kanaal, emi odude ke center.

anọ na ụdị nke anomalies e ekenyela na 1891 Arnold Chiari, iji nke a nhazi ọkwa ruo ugbu a.

Arnold-Chiari syndrome ụdị bụ nhichapụ posterior fossa okpokoro isi na gburugburu owuwu na spinal kanaal dị ukwuu ala karịa larịị nke foramen Magnum. Na nke abụọ ụdị anomaly bụ ugbu caudal na eze nke medulla oblongata, ala akụkụ nke irighiri na IV ventricle, na ezi mgbe ịzụlite hydrocephalus. Arnold-Chiari syndrome bụ a atọ ụdị dị nnọọ obere, n'ihi na ọ e ji ntụ dechapụ niile ụbụrụ owuwu nke posterior fossa. The anọ ụdị na-anọchi anya cerebellar hypoplasia. The anọ na atọ na ụdị anomalies ọtụtụ mgbe, ọ na-emegide ndụ.

Na 80% nke ikpe, Arnold-Chiari syndrome na-chọpụtara na syringomyelia - daa ọrịa nke ọgidigi azụ ji mmepe nke cysts nke ọgidigi azụ, nke na-kpụrụ nhichapụ posterior fossa mkpakọ nke okpokoro isi na cervical n'ógbè ọgidigi azụ. Ọnụnọ ndị cysts-eduga ná mmepe nke na-aga n'ihu myelopathy.

Arnold-Chiari syndrome: Mgbaàmà

The adakarị picture nke ọrịa e ji ndị na-esonụ mgbaàmà:

• mgbu n'olu na occipital ebe, nke enwekwu mgbe Nje ma ọ bụ coughing;
• ẹsụhọde okpomọkụ na ihe mgbu uche, nakwa dị ka nke elu ụkwụ muscle ume;
• N'ịdị spasticity;
• dizziness, na-ada mbà, unsteadiness, kawanye njọ site mbịne gị isi, na-ebelata visual acuity na àgwà nke ọhụụ.

Na oké ikpe mgbaàmà mmezi ndị a na-adịghị adịte cessation nke na-eku ume (apnea), na-agwụ ike nke pharyngeal mpiaji na ngwa ngwa, ịsụ mmegharị anya.

Mgbe ụfọdụ, Arnold Chiari anomaly nwere ike nwere ihe ọ bụla mgbaàmà na-achọta kpamkpam site ohere n'oge na-achọpụta ọrịa usoro. Diagnosis nke ọrịa a rụrụ iji MR Onyonyo na agbakọrọ tomography na atọ akụkụ nwughari nke cervical vertebrae, na occipital ọkpụkpụ.

Arnold-Chiari syndrome: Ọgwụgwọ

E Kenyere ọgwụgwọ na-adabere na ndidi na ịdị njọ nke ọrịa. Na ndị a bụ isi ihe mgbaàmà dị ka isi ọwụwa na ndị ọzọ ụdị ihe mgbu, gị na dọkịta nwere ike ikwu na ihe mgbu ọgwụ na mgbe ego-acha ọkụ. Ụfọdụ ọrịa kọrọ efe mgbe ewere mgbochi mkpali ọgwụ ọjọọ nwere ike igbochi ma ọ bụ ọbụna na-egbu oge ịwa.

Ọtụtụ mgbe, ọgwụgwọ, ndị dọkịta na-ahọrọ na-eji abnormalities surgically. The ọrụ bụ ka ọ kwụsị progression nke mgbanwe nke ọkpụkpụ azụ na ụbụrụ Ọdịdị na stabilization nke ihe mgbaàmà. Mgbe arụ a nke ọma ịwa vmeschatelstva budata mbelata nsogbu na ọgidigi azụ na cerebellum, nakwa dị ka na-agbake na-CSF outflow. E nwere dị iche usoro nke arụmọrụ na-adabere na-enwe hydrocephalus. The nkezi oge nke ịwa ahụ bụ onye na ọkara ka awa abụọ, na mgbake oge na-ewe ise ka ụbọchị asaa.