Yintoni pathology ekuthiwa Weber syndrome? Weber syndrome: oonobangela, iimpawu kunye nonyango

Sturge-Weber syndrome - isifo ilifa ngoweqela of alternating Hemiplegia. It idibanisa ngokoyiswa kwimithambo- cranial ukusuka eziko sifo imisebenzi sensory kunye neyezihlunu kwelinye icala. Weber syndrome ebizwa luvo syndrome, apho umnqonqo okanye ezonakeleyo elakha ngayo luvo oculomotor. Iimpawu imifuziselo sifo zibonakaliswa ebusweni, ngecala obuselukhambeni apho ptosis, mydriasis, strabismus, Hemiplegia engundoqo contralateral, ukushwabana izihlunu kobuso kunye nolwimi.

Weber syndrome: Iimpawu

Weber syndrome sisifo apho kuvela kwimimandla echaphazelekayo iimpahla iliso ulusu angiomas, angioma saphawula le conjunctiva; na uphuhliso olunokubakho esweni, glaucoma, kunye lamabutho semehlo.

kwakhiwa angioma multiple, nabo esebenza nolusu nezihlunu ezingqongileyo, wabonisa iimpawu luvo iimpawu kwi mater pia. Isikakhulu sifo sichaphazela indawo kwemihlathi yangasentla neliso luvo trigeminal. Ukonakala nobuchopho iqokobhe athambileyo isenokuba nokuba kwicala elinye kuphela, okanye zichaphazela zombini.

Ummandla uchaphazelekayo womGaqo-ubuso ngamabala ezithile umbala obomvu, kunye, ukongeza luvo, isifo zingachaphazela amalungu zangaphakathi.

Weber syndrome luphawulwa mpawu zilandelayo:

• Le mifanekiso MRI okanye CT scan - uboniso leptomeningeal angioma.
• basabela Convulsive.
• ukukhubazeka engqondweni (idiocy, ukukhubazeka engqondweni).
• Ukwanda uxinzelelo intraocular.
• Ukuswela umbono.
• Hemiparesis.
• Yome.
• Angiomas (isikhumba ebomvu wayifulela amabala nemithambo).

uhlobo syndrome Weber

Chonga iintlobo ezininzi ezinkulu. Echazwe ngentla apha ibonisa isifo - Weber syndrome. Ifoto ibonisa inkangeleko yomntu nokubandezeleka kulo disorder. Isifo wohlulwe:

• Schirmer-syndrome - Uphuhliso yokuqala ukunyanga iglaucoma agalelwa kunye ocular izenzo angioma cutaneous.
• Milles-syndrome - hemangioma yophuhliso iliso ngaphandle wokujoyina kunye ekuqaleni iimpawu neglaucoma angiomas eluswini kunye namehlo.
• Knud-Rrubbe-syndrome - kubonisa angioma entsefalotrigeminalnoy.
• Weber- Dumitri-syndrome - kubonisa sokuxhuzula, ukuxhuzula, ukulibaziseka yophuhliso, hemihypertrophy.
• Jahnke-syndrome - angioma ulusu, iindawo ebuchotsheni.
• Loford-syndrome - angioma ukhozo lweliso ngaphandle sikrini.

Weber syndrome: Oonobangela

Unobangela sifo kuxhomekeke yokuba ngexesha uphuhliso umbungu umonakalo ezimbini layers intsholongwane: ectoderm kunye mesoderm. Uphuhliso lwabantwana kule sifo khona kakhulu. Esi sifo sisasazwa ikakhulu alleles elibalaseleyo, kodwa kuyenzeka kunye nelifa sofuzo.

Esi sifo ukuphuhlisa kwi osadaleka khulelwa oko kunefuthe elibi. Ezi ziquka:

• Ukutshaya ngexa lokukhulelwa, ingakumbi xa usaqala.
• Ukusetyenziswa kotywala.
• Sebenzisa iziyobisi.
• Ukunxila yabafazi abamithiyo etiologies ezahlukeneyo.
• Ukusetyenziswa kwamalweyile ngendlela iziyobisi.
• izifo ezikumalungu ezifunyenwe ngethuba lokukhulelwa.
• usuleleko intrauterine.
• Disorder kwi nethe Umama okhulelweyo (hypothyroidism).

Amaxesha amaninzi, ukuze kuhlaselwa sesi sifo sihlasela yofuzo. Weber syndrome Awunokosulelwa kubomi bemihla ngemihla.

lokufunyaniswa kwesifo

Esi sifo axilonge wabonakala isici. Inxalenye kobuso yomguli usekuhleni utshintsho angiomatous, ukwanda koxinzelelo iliso kunye nokwanda lweliso lakho kunokwenzeka, ngaba sokuwa. Ukuba kukho urhano ka Weber syndrome, isifo kwaye afumane unyango yenziwa ngoogqirha ezininzi: zamehlo, luvo, epileptologist, wesikhumba.

Ukucacisa uxilongo lufuna uviwo X-ray. Zakuba zifikile imifanekiso kungabonwa calcification le neuron. Itomography ngekhompyutha inika nokuba epheleleyo ngakumbi umfanekiso kwimimandla echaphazelekayo. Le mifanekiso usibambe MRI, uyakwazi ukubona ukuxozwa le neuron, ukuphelelwa kunye yoma le nto emhlophe. Hardware uxilongo kufuneka kwakhona ibe nokungasetyenziswa kwezinye, izifo eziyingozi ngokulinganayo: ithumba ebuchotsheni, ithumba ezithambileyo cysts zeethishu ebuchotsheni.

A indima enkulu ngenxa uxilongo echanekileyo lidlala EEG ume, kuba loo nto yenza kube lula ukufumanisa umsebenzi neemvakalelo bioelectric ebuchotsheni, ngexesha ukufumanisa ukuxhuzula. Uninzi lwabantu abanengxaki onaso ka Weber, ezi sokuwa. nesilinganiso Kwakhona olwenziwe xi intraocular, ngumhlaba lwembono, ophthalmoscopy, AV ukuskena - kokuqeshwa unyango lwamehlo.

Weber Syndrome Treatment

Weber syndrome okwangoku an unyango olusebenzayo akwenziwanga kunye nonyango ejoliswe isiqabu inokuphumela nokuphucula umgangatho wobomi. Isigulane yabelwe unyango anticonvulsant kunye amayeza awohlukeneyo:

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

Kaninzi isigulane akaphenduli kwimpatho sokuwa, ngoko ke ugqirha aqeshwe ukusetyenziswa kweziyobisi ezininzi. Ukuba ngaba uncedo usilenaaya unyango, ngenxa yobungqina a neurosurgeon kufuneka yabelwe unyango ngotyando.

uxinzelelo intraocular ephakamileyo (glaucoma) iphathwa kunye ngamathontsi ezikhethekileyo, ukunciphisa secretion of uburharha zolwelo. La machiza aquka "timolol", "Alfagan", "Azopt," "Dorzolamide". Noko ke, le uthomalaliso elondoloza kunokuba kakuhle kakhulu, kwaye ke kuphela ukhetho yi uqhaqho: izigulane ndenziwa trabeculectomy okanye trabekulotomiyu.

Imiphumela kanye nobunzima

Weber Syndrome - isifo esiyingozi. Kwimeko unyango endala okanye ngotyando zanelisiwe kwaye imeko yomguli akubi ngcono, anikwe ALS kunokuba abahluphekayo. Ongalawulekiyo nabanesithuthwane syndrome kunokukhokelela sengqondo esixhalabisayo, ukukhubazeka engqondweni, ubumfama, ukwanda amathuba okuba nestroke.

Uthintelo kwesifo

Ngaphandle kwento yokuba isifo ilifa, amanyathelo othintelo wenziwe umfazi okhulelweyo, ziyakwehlisa ngokubonakalayo amathuba nokuphuhlisa sifo. Le misebenzi ibandakanya:

• Ukwala nemikhwa emibi (ingakumbi ngexesha lokukhulelwa).
• Ukugcina ngendlela echanekileyo yaye isempilweni. iin- rhoqo esemsebenzini, ulale esempilweni.
• izidlo olululo uphuphu. Ukunyuka ekutyeni kunye nokudla nefayibha. Musa ukusebenzisa iimveliso semi-igqityiwe kunye nokutya eqhotsiweyo.
• Ngexesha yobhaliso ukukhulelwa kunye notyelelo ukuya kugqirha ngexesha elicwangcisiweyo.
• Ukusetyenziswa kweziyobisi kuphela kagqirha eyenzelwe yona.

Weber syndrome - isifo enqabileyo kwaye iyingozi, apho kubonakala ngqo kwi umgangatho wobomi babantu. oogqirha Trust, uphethwe ngexesha kunye sicatshulwa yonyango ezimiselweyo ukutshintsha ubomi babantu. Kwakhona kubalulekile ukuba esi sifo uthathe indawo yawo kwaye hayi ukuba ukusebenzisa iziyobisi ngaphandle kagqirha.