Chii zvokurwara anonzi Weber chirwere? Weber chirwere: zvinokonzera, zviratidzo uye kurapwa

Sturge-Weber chirwere - ane nhaka chirwere kungova boka rakatumbuka hemiplegia. Chine pokukundwa ari cranial tsinga pachoto uye nyonganyonga kutsinga dzinouya uye injini mashandiro nokune rumwe rutivi. Weber chirwere inonzi chetsinga urwere, umo musana kana kukuvaradzwa musimboti wenyika oculomotor etsinga. Typical zviratidzo chirwere anozivikanwa pachiso, kurutivi pachoto ikoko ptosis, mydriasis, strabismus, musimboti contralateral hemiplegia, kuoma tsandanyama chiso uye rurimi.

Weber urwere: Signs

Weber chirwere chirwere umo kuonekwa munzvimbo sei ganda angiomas, angioma vakacherechedzawo ziso midziyo conjunctiva; zvinogoneka Kugadzirwa tsanga, yemaziso, uye retinal yokuzviparadzanisa.

kuumbwa vakawanda angioma, uko anoshanda ganda uye dzakapoteredza munyama, akaratidza maitiro chetsinga zviratidzo zviri pia mai. Most kazhinji chirwere kunokanganisa nzvimbo rokumusoro rushaya uye ziso trigeminal tsinga. Uropi nyoro goko vangava chete rumwe, kana kukanganisa zvose.

The zvakakanganisa nzvimbo chiso inotaurwa dzakananga makwapa akasvibirika dzvuku ruvara, pamwe, kuwedzera kune tsinga, chirwere kunogona nhengo dzomukati.

Weber chirwere kunoratidzwa anotevera zviratidzo:

• Mifananidzo MRI kana CT tarisei - display leptomeningeal angioma.
• Convulsive kuita.
• Mental retardation (idiocy, mupfungwa retardation).
• Yakawedzera intraocular kumanikidzwa.
• Kushayikwa maonero.
• Hemiparesis.
• Atrophy.
• Angiomas (dzvuku ganda akafukidzwa vascular makwapa akasvibirika).

Weber urwere marudzi

Ziva mhando huru dzakawanda. Pamusoro apa inoratidza chirwere - Weber syndrome. Photo chinoratidza Zvinoonekwa munhu chirwere ichi. The chirwere rakakamurwa kuva:

• Schirmer-chirwere - mangwanani kukura yemaziso uye ocular kuratidzwa cutaneous angioma.
• Milles-chirwere - hemangioma ziso kukura pasina kubatana mangwanani yemaziso zviratidzo angiomas paganda uye meso.
• Knud-Rrubbe-chirwere - nokuratidzwa angioma entsefalotrigeminalnoy.
• Weber- Dumitri-chirwere - nokuratidzwa nepfari, nezvipusha, kunonoka kukura, hemihypertrophy.
• Jahnke-chirwere - angioma ganda, nzvimbo muuropi.
• Loford-chirwere - angioma yeziso pasina magnification.

Weber urwere: Zvinokonzera

Mhaka hosha iri chokwadi kuti panguva kukura mudumbu zvakakuvadza maviri utachiona akaturikidzana: ectoderm uye mesoderm. Pokumuka Vana chirwere ichi apo neapo chaizvo. The chirwere iri wakapiwa kushandisa hukuru alleles, asi zvinoitika uye recessive nhaka.

The chirwere anogona kuva ari mudumbu kana pamuviri pacho inokanganisa. Idzi dzinosanganisira:

• Kusvuta uine pamuviri, kunyanya mu tanga.
• Kushandiswa doro.
• Kushandisa zvinodhaka.
• Kudhaka of pamuviri vakadzi etiologies siyana.
• Kushandiswa kusingadzoreki zvinodhaka.
• Dzokubereka Zvirwere vawana panguva yepamuviri.
• Intrauterine chirwere.
• Nyonganyonga iri vanepamuviri mai muviri (hypothyroidism).

Kakawanda, kukudziridza chirwere chete kunokanganisa nhodzera. Weber chirwere hahugoni utachiona muupenyu hwezuva nezuva.

chirwere chirwere

Maperembudzi chirwere ne noruzhinji kuratidzwa. Part chiso murwere ari pachena angiomatous kuchinja, vakawedzera ziso kumanikidza ane zvichibvira nokuwedzera yeziso, muri zvipusha pfari. Kana paine fungidziro pamusoro Weber urwere, chirwere uye kurapwa vari kuitwa navanachiremba akawanda: ophthalmologist, wetsinga, epileptologist, dermatologist.

Kujekesa nechirwere inoda X-ray kuzviongorora. Pamusoro okutambirwa mifananidzo inogona kuonekwa calcification pakati cerebral cortex. Computed tomography rinopa kunyange zvizere mufananidzo vawirwa nenjodzi. The zvifananidzo akabatwa MRI, anogona kuzviona rive dete pakati cerebral cortex, degeneration uye atrophy pamusoro chena nyaya. Hardware Diagnostics anofanirawo kuva achisiya vamwe, zvirwere zvakafanana ngozi: rouropi, pute nyoro uropi cysts.

A rakakura basa nokuda zvakarurama zvokuongorora chinoita eeg kunhaka, sezvo zvinoita kuti tikwanise kuona basa bioelectric nomunhu muuropi, nenguva rokuongorora netsviyo. Vazhinji vanhu vachitambura kubva urwere pakati Weber, muri zvipusha pfari. Also inoitwa nokuyerwa intraocular kumanikidzwa, munda maonero, ophthalmoscopy, AV kushanda pakuvheneka - kuti kugadzwa ophthalmic kurapwa.

Weber Syndrome Treatment

Weber chirwere iri pari anobudirira kurapwa haina kuitwa uye kurapwa nechinangwa chiratidzo zororo uye nani huri upenyu. The murwere basa anticonvulsant kurapa pamwe mishonga yakasiyana:

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

Kazhinji murwere haateereri pakurapa zvipusha nezvipusha, zvino chiremba anogona anogadzwa kushandisa zvinodhaka vakawanda. Kana musingadi kubatsira usilenaaya yokurapa, maererano neuchapupu ane neurosurgeon vangaendeswa kune kuvhiya kurapwa.

Yakakwirira intraocular kumanikidzwa (yemaziso) ikarapwa chaizvo madonhwe, kuderedza secretion pamusoro aqueous zvinosetsa. zvinodhaka izvi zvinosanganisira "timolol", "Alfagan", "Azopt," "Dorzolamide". Zvisinei, izvi kurapa kuchinja zvinogona va neudhadha chaizvo, uye ipapo chete nzira iri kuvhiyiwa: varwere vakaidzwa trabeculectomy kana trabekulotomiyu.

Consequences uye matambudziko

Weber Syndrome - ndiko chirwere ane ngozi. Kana kuchinja kana kuvhiya kurapwa vave akasangana uye murwere nemamiriro asina nani, akapiwa nani murombo fungidziro. Dzisingadzorwi zvipusha chirwere kungaita kuti dementia, retardation mupfungwa, upofu, mikana sitiroko.

Kudzivirira chirwere

Pasinei chokwadi kuti chirwere chacho chichigarwa nhaka, matanho kuitwa nomukadzi pamuviri, zvichaita kuderedza ngozi kuva chirwere. Zviitwa izvi zvinosanganisira:

• Kurambirwa tsika dzakaipa (kunyanya panguva yepamuviri).
• Kuramba akarurama uye utano mararamiro. Kakawanda inofamba yakachena mhepo, utano hope.
• Kwakakodzera paradzanisa zvokudya. Nokuwedzera zvokudya dzakakwirira faibha zvokudya. Usashandisa Semi-dzapedza zvinhu uye zvakakangwa chikafu.
• Panguva Chaiyo kunyoresa pamwe pamuviri uye kushanyira chiremba nenguva yakarongwa.
• Kushandisa zvinodhaka chete ayo aida mushonga.

Weber chirwere - isingawanzowanikwi uye ngozi chirwere, uko kunoratidzwa zvakananga pamusoro mararamiro evanhu. Trust vanachiremba, nenguva chirwere uye ndima ushandiswe kurapwa kunogona kuchinja upenyu hwavo. Zvinokoshawo haana kurega chirwere kutora vadzore uye kwete kushandisa zvinodhaka pasina mushonga.