ImpiloIzifo nemibandela

Yini zokugula ngokuthi Weber syndrome? Weber syndrome: izimbangela, izimpawu kanye nokwelashwa

Sturge-Weber syndrome - isifo ngofuzo bohlanga kushintshana hemiplegia. Sihlanganisa ukunqotshwa imizwa cranial eziko kanye disorder sezinzwa kanye motor imisebenzi ngakolunye uhlangothi. Weber syndrome ngokuthi zezinzwa syndrome, lapho emgogodleni noma i-nucleus ezilimele of emsipheni oculomotor. izimpawu ejwayelekile lesi sifo baziwa ebusweni, ohlangothini le khona ptosis, mydriasis, strabismus, emaphakathi hemiplegia contralateral, ukukhubazeka we imisipha ubuso nolimi.

Weber syndrome: Izimpawu

Weber syndrome ubulwelwe lapho avele abaningi babephephile ezindaweni ezithintekile izitsha iso isikhumba angiomas, angioma yaphawula we conjunctiva; ukuthuthukiswa kungenzeka esweni, ukufiphala kwamehlo kanye lamabutho ngwengwzi.

kwakhiwa angioma amaningi, okuyinto ngazo ziyasebenza isikhumba nezicubu ezizungezile, kukhombise ngokusobala izimpawu nobuntu zezinzwa kule Mater pia. Ngokuvamile lesi sifo sihlasela endaweni ongenhla futhi iso emsipheni trigeminal. Ukulimala kobuchopho igobolondo esithambile kungenzeka kunoma yiluphi uhlangothi olulodwa kuphela, noma zithonye.

Endaweni ethintekile ebusweni imbozwe nezimagqabhagqabha ezithile umbala obomvu, ne, ngaphezu isimiso sezinzwa, isifo kungathinta izitho zangaphakathi.

Weber syndrome libhekene zimpawu ezilandelayo:

  • Izithombe MRI noma CT scan - isibonisi leptomeningeal angioma.
  • ukuphendula Convulsive.
  • retardation kwengqondo (idiocy, retardation engqondo).
  • Ukwandiswa ingcindezi intraocular.
  • Ukuntula umbono.
  • Hemiparesis.
  • Yoma.
  • Angiomas (isikhumba obomvu embozwe nezimagqabhagqabha semithambo).

Weber zinhlobo syndrome

Thola izinhlobo eziningana ezinkulu. Ezichazwe ngenhla likhombisa isifo - Weber syndrome. Photo libonisa ukubukeka umuntu ukuphathwa yilesi sifo. Lesi sifo uhlukaniswe:

  • Schirmer-syndrome - ukuthuthukiswa okuqala e-glaucoma kanye ocular kuwukubonakaliswa angioma cutaneous.
  • Milles-syndrome - hemangioma ukuthuthukiswa iso ngaphandle kokujoyina izimpawu glaucoma ekuseni angiomas esikhumbeni futhi amehlo.
  • UKnud-Rrubbe-syndrome - ibonakalisa angioma entsefalotrigeminalnoy.
  • Weber- Dumitri-syndrome - ibonakalisa sokuwa, ukuquleka, ukubambezeleka zentuthuko, hemihypertrophy.
  • Jahnke-syndrome - angioma kwesikhumba, izindawo zobuchopho.
  • Loford-syndrome - angioma inhlamvu yeso ngaphandle kokukhulisa.

Weber syndrome: Izimbangela

Imbangela yale isifo itholakala lokuthi ukuthi phakathi ukuthuthukiswa umbungu ezilimele ezimbili izingqimba lwegciwane: yi-ectoderm ne-mesoderm. Ukuthuthukiswa Abantwana balesi sifo oqabukelayo kakhulu. Lesi sifo kuthiwa ngobulili ngokuyinhloko alleles ovelele, kodwa kwenzeka futhi ifa esikhubazayo.

Lesi sifo ingaqala ku-fetus khulelwa ke inomphumela omubi. Lezi zihlanganisa:

  • Ukubhema ngesikhathi sokukhulelwa, ikakhulukazi kusaqalwa.
  • Ukusetshenziswa kotshwala.
  • Sebenzisa izidakamizwa.
  • Lokudakwa abesifazane abakhulelwe etiologies ezahlukene.
  • Ukusetshenziswa engalawuliwe izidakamizwa.
  • izifo zangasese aluthola ngesikhathi sokukhulelwa.
  • ukutheleleka Intrauterine.
  • Kwemikhuba ku okhulelwe umama umzimba (i-hypothyroidism).

Ngokuvamile, ukuthuthukisa isifo kuphela kuthinta ufuzo. Weber syndrome angeke alithole igciwane ekuphileni kwansuku zonke.

sitholakele isifo

Lesi sifo ngomshini ukubonakaliswa isici. Ingxenye ubuso lesiguli echayeke izinguquko angiomatous, kukhuphuke iso ingcindezi nokwanda kwentshiseko okungenzeka yeso, kukhona izinhlobo zesifo sokuwa. Uma kukhona izinsolo Weber syndrome, ukuxilongwa kanye nokwelashwa yenziwa odokotela eziningana: ophthalmologist, udokotela wezinzwa, epileptologist, wesikhumba.

Ukuze sicacise uphethwe kudinga-X-ray luhlolo. Phezu kokuthola izithombe kungabonwa calcification we- cortex cerebral. computed tomography kunikeza ephelele nakakhulu isithombe babephephile ezindaweni ezithintekile. Izithombe ethunjwa MRI, kungathola thinning ye-cerebral cortex, ukonakala kanye yoma sendaba emhlophe. Hardware ukuhlola kufanele futhi kube exclusion nezinye, izifo eziyingozi ngokulinganayo: ebuchosheni, ithumba ezithambile ubuchopho izicubu izinhlumba.

A indima enkulu uphethwe yini udlala Bheka eliyifa, njengoba kwenza kube nokwenzeka ukuba sithole umsebenzi inkanuko bioelectric ebuchosheni, ngesikhathi ukuxilonga isithuthwane. Iningi labantu abaphethwe sifo of Weber, kukhona izinhlobo zesifo sokuwa. nesilinganiso Futhi olwenziwa yokucindezelwa intraocular, emkhakheni buka, ophthalmoscopy, AV ukuskena - nokuqokwa ukwelashwa amehlo.

Weber Syndrome Ukwelashwa

Weber syndrome okwamanje ukwelashwa okuphumelelayo akuyona eyenziwa kanye nokwelashwa kuhloswe impumuzo uphawu nezinga lokuphila ngcono. Isiguli inikezwa yokwelapha anticonvulsant imithi ehlukene:

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

Kaningi isiguli akasabeli ekwelapheni zesifo sokuwa, udokotela bese angaqokwa njengomhloli ukusetshenziswa kwezidakamizwa eziningi. Uma ungenalo asize usilenaaya yokwelapha, ngo ubufakazi udokotela ohlinza izinzwa ingase yabelwe ukwelashwa kuhlinzwa.

Isigqugquzeli ingcindezi intraocular (glaucoma) ziphathwe amathonsi ekhethekile, ukunciphisa uketshezi of amahlaya aqueous. Le mithi ihlanganisa "timolol", "Alfagan", "Azopt," "Dorzolamide". Nokho, lokhu yokwelapha olandelanayo kungaba engaphumeleli neze, bese ukuphela kwesixazululo ukuhlinzwa: iziguli kwavele trabeculectomy noma trabekulotomiyu.

Imiphumela kanye nezinkinga

Weber Syndrome - yisifo esiyingozi. Endabeni ukwelashwa ngoludala noma kuhlinzwa kuhlangatshezwene nazo futhi isimo lesiguli akubi ngcono, uma unikezwe Izibikezelo kunalokho abampofu. Engalawuliwe syndrome nabanesithuthwane kungaholela komqondo, retardation engqondo, ubumpumputhe, sengozini enkulu yokufa uhlangothi.

Ukuvimbela lesi sifo

Naphezu kweqiniso lokuthi lesi sifo sitholakala ngofuzo, izinyathelo zokuvimbela eyenziwa okhulelwe zizonciphisa ingozi yokuba lesi sifo. Lemisebenti zihlanganisa:

  • Ukunqatshwa imikhuba emibi (ikakhulukazi lezo ezimithi).
  • Ukulondoloza yokuphila efanele futhi unempilo. izizinda njalo emoyeni opholile, ukulala okunempilo.
  • Proper yokuhlukanisa ukudla. Ukwenyuka ekudleni kwabo okusezingeni eliphezulu ukudla fibre. Ungasebenzisi imikhiqizo ephuma kabili isiqediwe futhi ukudla okuthosiwe.
  • ukubhaliswa Esifike Ngesikhathi ukukhulelwa kanye nokuya kudokotela ngesikhathi esibekiwe.
  • Ukusetshenziswa kwezidakamizwa kuphela kadokotela wawo ahlose ukuba usetshenziswe.

Weber syndrome - isifo ezingavamile futhi iyingozi, okuyinto ngqo ibonakale izinga lokuphila komuntu. Trust odokotela, uphethwe esifike ngesikhathi futhi ukudlula ukuphathwa ebekiwe Ungashintsha ukuphila kube ngcono. Kubalulekile futhi ukuba singavumeli lesi sifo busebenze hhayi ukusebenzisa izidakamizwa ngaphandle kadokotela.

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