Sturge-Weber Syndrome yog ib hom kab mob uas muaj nyob hauv cov pab pawg ntawm cov kab mob sib txawv. Nws ua ke kev puas tsuaj rau cov hlab ntsha cranial nyob rau sab ntawm kev ua kom pom tseeb thiab muaj kev cuam tshuam ntawm kev hnov qab thiab lub cev muaj zog ua haujlwm ntawm sab nraud. Weber's Syndrome yog ib hom kab mob neurological uas lub hauv paus lossis lub hauv paus ntawm cov hlab ntsha oculomotor puas. Cov yam ntxwv tshwm sim ntawm tus kab mob yog tshwm sim ntawm lub ntsej muag, nyob rau sab ntawm lub ntsej muag muaj ptosis, mydriasis, strabismus, contralateral central hemiplegia, tuag tes tuag taw ntawm lub ntsej muag thiab tus nplaig.
Weber Syndrome: kos npe
Weber's syndrome yog ib yam kab mob uas angiomas tshwm sim ntawm thaj chaw cuam tshuam ntawm daim tawv nqaij, angiomas ntawm cov hlab ntsha ntawm lub qhov muag, conjunctiva kuj tau pom; Kev loj hlob ntawm cataracts, glaucoma, thiab retinal detachment.
Ntau yam angiomas daim ntawv ntawm pia mater, uas tseem kis mus rau ntawm daim tawv nqaij ntawm lub ntsej muag thiab cov ntaub so ntswg uas nyob ib sab, thiab cov tsos mob ntawm lub paj hlwb tshwm sim. Feem ntau, tus kab mob cuam tshuam rau thaj tsam ntawm lub puab tsaig sab sauv thiabophthalmic trigeminal paj hlwb. Kev puas tsuaj rau lub plhaub mos ntawm lub hlwb tuaj yeem tsuas yog ntawm ib sab, lossis cuam tshuam rau ob qho tib si.
Qhov cuam tshuam ntawm lub ntsej muag yog npog nrog cov pob liab liab, thaum ntxiv rau lub paj hlwb, tus kab mob kuj tuaj yeem cuam tshuam rau hauv nruab nrog cev.
Weber syndrome yog tus cwj pwm los ntawm cov tsos mob hauv qab no:
- MRI lossis CT cov duab qhia pom qhov tshwm sim ntawm leptomeningeal angioma.
- Cov tshuaj tiv thaiv kab mob.
- Mental retardation (idiocy, hlwb retardation).
- Npaj siab intraocular.
- Vision deficit.
- Hemiparesis.
- Hemiatrophy.
- Angiomas (cov tawv nqaij npog nrog cov pob liab liab).
Ntau yam ntawm Weber's Syndrome
Muaj ntau ntau yam tseem ceeb. Cov kab mob piav qhia saum toj no - Weber's syndrome. Daim duab qhia tias tus neeg raug kev txom nyem los ntawm tus kab mob no zoo li cas. Tus kab mob no tau muab faib ua:
- Schirmer-syndrome - kev loj hlob ntawm glaucoma thaum ntxov nrog tshwm sim ntawm daim tawv nqaij thiab qhov muag angioma.
- Milles-sydrome - kev loj hlob ntawm hemangioma ntawm lub qhov muag yam tsis muaj qhov sib ntxiv ntawm glaucoma thaum ntxov nrog kev tshwm sim ntawm angioma ntawm daim tawv nqaij thiab hauv qhov muag.
- Knud-Rrubbe-syndrome - ib qho kev tshwm sim ntawm encephalotrigeminal angioma.
- Weber-Dumitri-sydrome - ib qho kev tshwm sim ntawm qaug dab peg, qaug dab peg, kev loj hlob qeeb, hemihypertrophy.
- Jahnke-syndrome - angiomas ntawm daim tawv nqaij, thaj chaw ntawm lub hlwb.
- Loford syndrome - angiomas ntawm lub qhov muag tsis muaj nws qhov loj.
SyndromeWeber: Vim li cas
Yog vim li cas rau qhov tshwm sim ntawm tus kab mob yog thaum lub sij hawm kev loj hlob ntawm tus me nyuam hauv plab, ob txheej kab mob puas lawm: lub ectoderm thiab mesoderm. Kev loj hlob ntawm tus kab mob no nyob rau hauv cov me nyuam yog heev episodic. Tus kab mob kis tau los ntawm cov tseem ceeb alleles, tab sis kuj tseem muaj cov qub txeeg qub teg.
Tus kab mob tuaj yeem tshwm sim hauv tus menyuam hauv plab yog tias thaum cev xeeb tub nws cuam tshuam tsis zoo. Cov no suav nrog:
- Kev haus luam yeeb thaum cev xeeb tub, tshwj xeeb tshaj yog thaum cev xeeb tub.
- Pob cawv.
- Kev siv tshuaj.
- Kev qaug rau tus poj niam cev xeeb tub ntawm ntau yam etiologies.
- Kev siv tshuaj tsis raug cai.
- Kev sib deev kis tau thaum cev xeeb tub.
- kab mob hauv lub cev.
- Metabolism disorder in expectant niam (hypothyroidism).
Ntau zaus, tsuas yog heredity cuam tshuam txoj kev loj hlob ntawm tus kab mob. Weber syndrome tsis tuaj yeem cog lus hauv lub neej zoo tib yam.
Kev kuaj kab mob
Tus kab mob no kuaj tau los ntawm cov yam ntxwv tshwm sim. Ib feem ntawm tus neeg mob lub ntsej muag yuav raug hloov pauv angiomatous, nce qhov muag siab nrog qhov ua tau nce hauv pob qhov muag, muaj qaug dab peg. Yog tias muaj qhov tsis txaus ntseeg ntawm Weber's syndrome, kev kuaj mob thiab kev kho mob tau ua los ntawm ntau tus kws kho mob: tus kws kho qhov muag, kws kho mob hlwb, kws kho mob epilepologist, kws kho mob dermatologist.
kev kuaj X-ray yog qhov tsim nyog kom paub meej qhov kev kuaj mob. Nyob ntawmCov duab tshwm sim tuaj yeem pom calcification ntawm cerebral cortex. Kev suav tomography muab ib daim duab ntau dua ntawm cov chaw cuam tshuam. MRI scans tuaj yeem pom qhov thinning ntawm cerebral cortex, degeneration, thiab atrophy ntawm cov teeb meem dawb. Kev kuaj mob kho vajtse kuj tseem tsim nyog kom tsis suav nrog lwm yam, tsis muaj qhov txaus ntshai, kab mob: mob hlwb, abscesses ntawm cov nqaij mos ntawm lub hlwb, cysts.
Electroencephalography plays lub luag haujlwm tseem ceeb hauv kev kuaj mob kom raug, vim nws ua rau nws muaj peev xwm txiav txim siab txog kev ua haujlwm ntawm bioelectric impulses hauv lub hlwb txhawm rau txhawm rau kuaj mob qaug dab peg hauv lub sijhawm. Feem ntau cov neeg uas muaj Weber syndrome muaj qaug dab peg. Lawv kuj ntsuas qhov muag siab, pom kev pom, pom kev pom, AV scanning - rau kev teem caij kho qhov muag.
Kev kho mob ntawm Weber's syndrome
Weber's syndrome tam sim no tsis muaj kev kho mob zoo, thiab kev kho tsis tu ncua yog txhawm rau txo cov tsos mob thiab txhim kho lub neej zoo ntawm tus neeg. Tus neeg mob tau txais kev kho mob anticonvulsant siv ntau yam tshuaj:
- "Depakin".
- Y"Carbamazepine".
- "Keppra".
- "Topiramate".
- "Finlepsin".
Ntau zaus tus neeg mob tsis teb rau kev kho mob qaug dab peg, tom qab ntawd tus kws kho mob tuaj yeem sau ntawv siv ntau yam tshuaj. Yog tias kev kho kom zoo dua tsis pab, cesCov lus qhia ntawm tus kws kho mob neurosurgeon, kev kho mob phais yuav raug sau.
Qhov muag siab (glaucoma) yog kho nrog cov tee tshwj xeeb uas txo cov kua dej qhov muag. Cov tshuaj no muaj xws li "Timolol", "Alfagan", "Azopt", "Dorzolamide". Txawm li cas los xij, qhov kev saib xyuas kev noj qab haus huv no tuaj yeem ua tsis tau zoo, thiab tom qab ntawd qhov kev xaiv tsuas yog kev kho mob phais: tus neeg mob tau txais trabeculectomy lossis trabeculotomy.
Kev tshwm sim thiab teeb meem
Weber's Syndrome yog ib yam kab mob txaus ntshai heev. Yog hais tias kev kho mob tsis tu ncua lossis kev phais mob tsis tau txais txiaj ntsig thiab tus neeg mob tus mob tsis zoo tuaj, qhov kev cia siab tsis zoo yog muab. Kev tswj tsis tau tus kab mob vwm tuaj yeem ua rau dementia, mob hlwb, tsis pom kev, thiab muaj kev pheej hmoo siab mob stroke.
Kev tiv thaiv kab mob kev loj hlob
Txawm hais tias tus kab mob no yog los ntawm keeb kwm, kev tiv thaiv los ntawm tus poj niam cev xeeb tub yuav txo qis kev pheej hmoo ntawm tus kab mob. Cov dej num no suav nrog:
- Kaws cwj pwm phem (tshwj xeeb thaum cev xeeb tub).
- Ua kom lub neej zoo thiab noj qab nyob zoo. Nquag taug kev hauv huab cua ntshiab, pw tsaug zog zoo.
- Khoom noj khoom haus kom zoo. Nce hauv kev noj zaub mov ntawm cov zaub mov muaj fiber ntau. Tsis txhob noj cov zaub mov tiav thiab cov zaub mov kib.
- sau npe raws sijhawm thaum cev xeeb tub thiab mus ntsib kws kho mob raws sijhawm.
- Kev siv tshuaj ncaj qha nkaus xwbtus kws kho mob tshuaj.
Weber's syndrome yog ib yam kab mob tsawg thiab txaus ntshai uas cuam tshuam ncaj qha rau tib neeg lub neej zoo. Kev ntseeg siab hauv cov kws kho mob, kev kuaj mob raws sij hawm thiab kev ua tiav ntawm kev kho mob tuaj yeem hloov lub neej kom zoo dua. Nws tseem yog ib qho tseem ceeb kom tsis txhob cia tus kab mob no mus rau nws txoj kev kawm thiab tsis txhob siv tshuaj yam tsis muaj kws kho mob sau ntawv.
