Waa maxay cudurada loo yaqaan Weber syndrome? Weber syndrome: sababaha, astaamaha iyo daweynta

Sturge-Weber syndrome - cudur dhaxlay tirsan kooxda talantaalli ah qalal. Waxay isku daraa guuldaradii ee dareemayaasha cranial ka girgire iyo disorder hawlaha dareenka iyo dhaqdhaqaaqa dhinaca ka soo horjeeda. Weber syndrome yeedhay dareemayaasha syndrome, taas oo dhabarta ama xuduntii burburay ee dareemayaasha oculomotor ah. calaamadaha lagu garto cudurka loo aqoonsan yahay wajiga, dhinaca qoryaha waxaa ptosis, mydriasis, strabismus, qalal contralateral dhexe, curyaannimo oo ka mid ah muruqyada wejiga iyo carrabka.

Weber syndrome: Calaamadaha

Weber syndrome waa cudur ay u muuqdaan in degaannada ay dhibaatadu saameysey ee weelashii isha angiomas maqaarka, angioma sidoo kale lagu arkay of conjunctiva ah; horumarinta suurto galka ah ee caadka, glaucoma, iyo go'idda.

sameeyay angioma badan, kaas oo sidoo kale codsan in ay maqaarka iyo unugyada ku wareegsan, muujiyey astaamaha dareemayaasha sifo ee Mater Pia ah. Inta badan cudurka saamayn ku degaanka ee daanka sare iyo indhaha dareemayaasha trigeminal. Burburka maskaxda qolof jilicsan noqon karaa hal dhinac oo keliya, ama saamayn labadaba.

meesha ay dhibaatadu saameysey wajiga daboolan by jaamaha gaarka ah ee midabka cas, oo leh, marka lagu daro habka dareemayaasha jirka, cudurka saamayn ku yeelan karaan xubnaha gudaha.

Weber syndrome waxa lagu gartaa calaamadaha soo socda:

• sawirada MRI ama CT scan - bandhigay angioma leptomeningeal.
• falcelin la marsiiyo.
• dhimaal maskaxeed (idiocy, waxyeello maskaxda).
• cadaadiska intraocular kordhay.
• Deficiency of view.
• Hemiparesis.
• Atrophy.
• Angiomas (maqaar cas oo ku dednaa jaamaha vascular).

noocyada syndrome Weber

Aqoonso dhowr noocyo waaweyn. kor lagu sharaxay waxay muujinaysaa cudurka - Weber syndrome. Photo muujinaysaa eegno qofka ka cabanaya cudur this. Cudurku wuxuu u qaybsan yahay:

• Schirmer-syndrome - horumarinta hore ee glaucoma iyo indhaha ah daliilka angioma cutaneous.
• Milles-syndrome - horumarinta isha hemangioma aan ku biiro oo leh calaamado glaucoma hore angiomas on maqaarka iyo indhaha.
• Knud-Rrubbe-syndrome - muujinta a of entsefalotrigeminalnoy angioma.
• Weber- Dumitri-syndrome - muujinta a of qallalka, qalal, dhac koritaan, hemihypertrophy.
• Jahnke-syndrome - angioma maqaarka, meelood oo maskaxda ka.
• Loford-syndrome - bu'da angioma aan weyneeyey.

Weber syndrome: Sababaha

Sababta oo ah cudurka been in xaqiiqda ah in inta lagu guda jiro horumarinta ilmaha caloosha ku burburay laba lakab jeermiga: ectoderm iyo mesoderm. horumarinta carruurta ee cudurka waa mid aad goosgoos ah. cudurka waxaa soo gudbiyo ugu horrayn by alleles adag, laakiin waxa dhacaya iyo dhaxal recessive.

Cudurku wuxuu horumarin karaa ilmaha caloosha ku jira kiiska ee uurka waxa ay leedahay saamayn xun. Kuwaas waxaa ka mid ah:

• Cabbidda sigaarka hooyada inta ay uurka leedahay, gaar ahaan marxaladaha hore.
• Isticmaalka khamriga.
• Isticmaal daroogada.
• Sarkhaan oo ka mid ah haweenka uurka leh ee etiologies kala duwan.
• Isticmaalka daroogada caasiyoobay.
• cudurada xubnaha taranka helay inta aad uurka leedahay.
• cudurka intrauterine.
• Disorder-shiid ee hooyada uurka leh ah (hypothyroidism).

Inta badan, si ay u horumariyaan cudurka wuxuu saameeyaa oo kaliya hiddo. Weber syndrome ma qaadi karaan nolol maalmeedka.

ogaanshaha cudurka

Cudurku waxa lagu baara daliilka sifo. Qayb ka mid ah wajiga qofka bukaanka ah la kulmin isbedel angiomatous, kordhay cadaadiska isha la koror suurto galka ah ee bu'da, waa suuxdintu. Haddii uu jiro shaki Weber syndrome, baaritaanka iyo daawaynta la fuliyaa by dhowr dhakhaatiirta: indhaha, neurologist, epileptologist, haragga.

Si loo xaqiijiyo cudurka u baahan yahay baaritaan X-ray. Marka la helo sawirada loo arki karaa kalshum kiliyaha cerebral. raajito loogu xisaabiyaa bixisaa xitaa ka dhameystiran sawir oo ka mid ah degaannada ay dhibaatadu saameysey. images The qabtay MRI, lagu ogaan karaa khafiifinta ah cerebral kiliyaha, xumaanshaha iyo atrophy xaalka cad. Baadhista xanuunada Hardware waa in sidoo kale noqon in laga saaro kale, cudurro si siman khatar ah, burada maskaxda, fiix unugyada maskaxda jilicsan malax.

doorka A weyn cudurka saxda ciyaara burburiyaha EEG, tan waxa ay taasi suurto gal ah si loo ogaado waxqabadka of falalkooda bioelectric ee maskaxda, in markii si loo baadho qalalka. Inta badan dadka ka cabanaya syndrome ee Weber, waa suuxdintu. cabiraada sidoo kale cadaadis intraocular, field of view, ophthalmoscopy, iskaanka AV - ballanta daaweynta indhaha.

Weber Syndrome Daaweynta

Weber syndrome ayaa hadda daaweynta ku ool ah looma fuliyo iyo daaweynta loogu talagalay gargaarka calaamad iyo in la hagaajiyo tayada nolosha. bukaan-socodka waxaa loo xilsaaray therapy anticonvulsant daawooyin kala duwan:

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

Marar badan bukaanka aanu ka jawaabin daaweynta suuxdintu, ka dibna dhakhtarka ayaa laga yaabaa in loo magacaabay isticmaalka daroogada kala duwan. Haddii aadan ku caawin usilenaaya therapy, by maragga of qofkale oo a waxaa laga yaabaa in loo xilsaaray in ay daaweynta qalliin.

cadaadiska intraocular kacsantahay (glaucoma) waxaa lagu daaweeyaa dhibcood gaar ah, yareynta dheecaan ee kaftan aqueous. daroogada waxaa ka mid ah "timolol", "Alfagan", "Azopt," "Dorzolamide". Si kastaba ha ahaatee, tani therapy muxaafidka ah waxay noqon kartaa mid aad u hawlqabad la'aan, ka dibna doorasho kaliya waa qalitaan: bukaanka maray trabeculectomy ama trabekulotomiyu.

Cawaaqibta iyo dhibaatooyin

Weber Syndrome - waa cudur khatar ah. In the case of daaweyn muxaafidka ah ama qalliin ayaa la kulmay iyo xaaladda bukaanka aan soo hagaagtay, siiyey saadaal ah halkii saboolka ah. Xakamaynin syndrome suuxdin waxay keeni kartaa in dementia, dhimaal maskaxeed, indho la'aan, halis dheeraad ah stroke.

Ka hortagga cudurka

In kasta oo xaqiiqda ah in cudurka uu yahay mid la iska dhaxlo, tallaabooyin ka hortag ah oo ay qof dumar ah oo uur leh la fuliyay, si weyn hoos u dhigi doontaa halista cudurka. Hawlahan waxa ka mid ah:

• Diidmada caadooyinka xun (gaar ahaan inta lagu guda jiro uurka).
• Joogtaynta nololeed sax ah oo caafimaad qaba. qaybaha soo noqnoqda ee hawada cusub, hurdo caafimaad leh.
• cunto kala habboon. kordhiyo cunto la cunnooyinka qamadidu ku sarreyso. Ha isticmaalin waxyaabaha semi-dhammeeyayna iyo cunto la shiilay.
• diiwaangelinta waqtigii uurka iyo booqashada dhakhtarka, wakhtigii loogu talagalay.
• Isticmaalka daroogada kaliya ee ay qoro loogu tala galay.

Weber syndrome - cudur dhif ah oo khatar ah, kuwaas oo si toos ah ka muuqataa on tayada nolosha aadanaha. dhakhaatiirta Trust, ogaanshaha waqtiga iyo marinka ee daaweynta qoray bedeli kartaa nolosha wanaagsan. Waxaa sidoo kale muhiim ah in aan si ha cudurka qaado koorsada iyo in aadan isticmaalin daawooyinka aan qoro.