Gịnị daa ọrịa na-akpọ Weber syndrome? Weber syndrome: akpata, mgbaàmà na ọgwụgwọ

Sturge-Weber syndrome - pụta ọrịa nke na ìgwè nke nwetụ hemiplegia. Ọ na-agwakọta e meriri ndị cranial akwara si n'ekwú na ọgba aghara sensory na moto ọrụ na-abụghị n'akụkụ. Weber syndrome akpọ akwara ozi syndrome, nke ọkpụkpụ azụ ma ọ bụ mebiri emebi ntọala nke oculomotor akwara. Ahụkarị mgbaàmà nke ọrịa na-ghọtara na ihu, n'akụkụ nke n'ekwú e ptosis, mydriasis, strabismus, Central contralateral hemiplegia, mkpọnwụ nke uru ahụ ndị dị na ihu na ire.

Weber syndrome: ịrịba ama

Weber syndrome bụ ọria nke na-egosi na ndị metụtara ebe nke anụ angiomas, angioma kwukwara anya nile nke conjunctiva; kwere omume mmepe nke cataracts, glaucoma, na retinal detachment.

kpụrụ multiple angioma, nke na-emetụtakwa ikpa na gburugburu anụ ahụ, na-egosipụta ihe e ji mara akwara ozi mgbaàmà na pịa mater. Ọtụtụ mgbe ọrịa na-emetụta ndị ebe nke elu agha na anya trigeminal akwara. Mmebi nke ụbụrụ soft shei nwere ike ma nanị otu akụkụ, ma ọ bụ na-emetụta ma.

Otụk ebe nke ihu kpuchie kpọmkwem patches nke acha ọbara ọbara, na, na mgbakwunye na nke ụjọ usoro, ọrịa nwere ike isi metụta esịtidem akụkụ.

Weber syndrome e ji ndị na-esonụ mgbaàmà:

• The foto MRI ma ọ bụ CT ike iṅomi - ngosi leptomeningeal angioma.
• Convulsive mmeghachi omume.
• Mental iberibe (idiocy,-eme iberibe).
• Mụbara intraocular nsogbu.
• Erughi nke ele.
• Hemiparesis.
• Atrophy.
• Angiomas (red akpụkpọ kpuchie vaskụla patches).

Weber syndrome umu

Chọpụta ọtụtụ isi iche. Akọwara n'elu na-egosi na ọrịa - Weber syndrome. Photo-egosi na anya nke a onye na-ata ahụhụ site na nsogbu a. The ọrịa ekewa:

• Schirmer-syndrome - n'oge mmepe nke glaucoma na ocular ngosipụta nke cutaneous angioma.
• Milles-syndrome - hemangioma anya development enweghị isonyere n'oge glaucoma mgbaàmà angiomas na akpụkpọ anya.
• Knud-Rrubbe-syndrome - a ngosipụta nke angioma entsefalotrigeminalnoy.
• Weber- Dumitri-syndrome - a ngosipụta nke Akwụkwụ na-adọ, ọdụdọ, otito na-egbu oge, hemihypertrophy.
• Jahnke-syndrome - angioma nke anụ ahụ, ebe nke ụbụrụ.
• Loford-syndrome - angioma nwa anya enweghị magnification.

Weber syndrome: akpatara

The akpata ọrịa dabeere n'eziokwu na n'oge mmepe nke nwa ebu n'afọ-abaghị uru abụọ ngidịrigi izizi: ektodamụ na mesodamụ. Mmepe nke ụmụaka ndị a na ọrịa bụ nnọọ sporadik. The ọrịa na-ebute site ná bụ isi ihe kasị alleles, ma ọ na-eme na recessive nketa.

The ọrịa nwere ike ịzụlite na nwa ebu n'afọ na ikpe nke na ime ya nwere a-ezighị ezi mmetụta. Ndị a gụnyere:

• Ịṅụ Sịga n'oge ime, karịsịa ná mmalite nkebi.
• The ojiji nke mmanya.
• Jiri nke ọgwụ ọjọọ.
• Mmenaanya nke ụmụ nwaanyị dị ime nke dị iche iche etiologies.
• The na-achịkwaghị achịkwa ojiji nke ọgwụ ọjọọ.
• Genital efe efe enwetara n'oge ime.
• Intrauterine ọrịa.
• Aghara na Nwanyị metabolism (hypothyroidism).

Ọtụtụ mgbe, ịzụlite ọrịa nanị na-emetụta si n'aka ruo n'aka. Weber syndrome-apụghị oria ná ndụ kwa ụbọchị.

nchoputa nke ọrịa

The ọrịa a chọpụtara site ndị e ji mara n'obiọma. Part nke ọrịa ihu n'anwụ angiomatous mgbanwe, mụbara anya mgbali na a ga-ekwe omume na-abawanye nke nwa anya, bụ akwụkwụ ọdịdọ. Ọ bụrụ na e enyo nke Weber syndrome, nchoputa na ọgwụgwọ na-rụrụ site na ọtụtụ ndị dọkịta: ophthalmologist, ọkà mmụta ọrịa akwara, epileptologist, dematọlọjist.

Iji dokwuo anya nchoputa na-achọ X-ray nyochaa. N'elu nnata nke foto nwere ike hụrụ calcification nke ụbụrụ cortex. Agbakọrọ tomography na-enye ihe ndị ọzọ zuru ezu na foto nke metụtara. The oyiyi weghaara MRI, nwere ike ịchọpụta na thinning nke ụbụrụ cortex, mbelata na atrophy nke na-acha ọcha okwu. Hardware nchọpụta nsogbu kwesịkwara na mwepu nke ọzọ, dokwara ize ndụ nke ọrịa: akpụ ụbụrụ, etuto soft ụbụrụ anụ ahụ cysts.

A nnukwu ọrụ maka ihe na-arịa na-arụ Lee nketa, ebe ọ na-eme ka o kwe omume iji chọpụta ọrụ nke bioelectric gboo na ụbụrụ, na oge nchọpụta Akwụkwụ na-adọ. Ihe ka ọtụtụ ná ndị mmadụ na-ata ahụhụ si syndrome nke Weber, bụ akwụkwụ ọdịdọ. Ọzọkwa ekenịmde n'ihe nke intraocular nsogbu, ubi nke na-ele, ophthalmoscopy, AV ịgụ isiokwu - na nhọpụta nke ophthalmic ọgwụgwọ.

Weber ọrịa Ọgwụgwọ

Weber syndrome a ugbu a irè ọgwụgwọ a na-adịghị rụrụ na ọgwụgwọ a na-iji na mgbaàmà enyemaka na mma àgwà nke ndụ. Onye ọrịa na-kenyere anticonvulsant ọgwụ dị iche iche na ọgwụ:

• "Depakine."
• "Carbamazepine."
• "Keppra".
• "Topiramate".
• "Finlepsin".

Ọtụtụ mgbe onye ọrịa dịghị aza ọgwụgwọ nke akwụkwụ ọdịdọ, mgbe ahụ, dọkịta nwere ike họpụtara dị ka ojiji nke multiple ọgwụ ọjọọ. Ọ bụrụ na ị na-enyere usilenaaya ọrịa, site na àmà nke a neurosurgeon nwere ike ikenye ịwa ahụ ọgwụgwọ.

Elu intraocular nsogbu (glaucoma)-emeso ya na pụrụ iche tụlee, na-ebelata secretion nke okenye ọchị. Ndị a ọgwụ ọjọọ eme ihe na-agụnye "timolol", "Alfagan", "Azopt," "Dorzolamide". Otú ọ dị, nke a mgbanwe ọgwụ pụrụ ịbụ nnọọ adighi ike, mgbe ahụ, nanị nhọrọ bụ ịwa ahụ: ọrịa wara trabeculectomy ma ọ bụ trabekulotomiyu.

Esi na ya pụta na nsogbu

Weber ọrịa - bụ a dị ize ndụ ọrịa. N'ihe banyere nke mgbanwe ma ọ bụ ịwa ahụ ọgwụgwọ e zutere na ọrịa na-arịa na-adịghị mma, nyere a kama ogbenye prognosis. Achịkwaghị achịkwa akwụkwụ syndrome pụrụ iduga mgbaka,-eme iberibe, kpuo ìsì, n'ihe ize ndụ nke ọrịa strok.

Prevention nke ọrịa

N'agbanyeghị eziokwu na ọrịa bụ butere n'aka, mgbochi rụrụ site a nwaanyị dị ime, ga budata belata ihe ize ndụ nke na-emepe emepe ọrịa. Ndị a na-eme na-agụnye:

• Ịjụ àgwà ọjọọ (karịsịa n'oge ime).
• Ịnọgide na-enwe ezi na ike ndụ. Ugboro n'ọnọdụ dị iche iche nke ọhụrụ ikuku, ike na-ehi ụra.
• Proper gbawara n'etiti nri. A na-abawanye na ihe oriri na elu eriri oriri. Unu na-eji ọkara okokụre ngwaahịa na e ghere eghe nri.
• Bịara n'Ezi Oge ndebanye na ime na-eleta ndị dọkịta na ndị oge ahụ.
• The iji ọgwụ ọjọọ eme naanị n'ihi na ya bu n'obi na ọgwụ.

Weber syndrome - a obere na-ize ndụ ọrịa, nke na-na-egosipụta na àgwà nke ndụ mmadụ. Trust dọkịta, na-adọ nchoputa na Itie nke kenyere ọgwụgwọ pụrụ ịgbanwe ndụ ka mma. Ọ dịkwa mkpa agaghị ekwe ka ọrịa iri ya N'ezie na ghara iji ọgwụ ọjọọ eme ihe na-enweghị a ọgwụ.