Tohu me te maimoatanga o Landau-Kleffner syndrome

Landau-Kleffner syndrome - riro te aphasia haurangi i roto i ngā tamariki. Kei te āhuatanga i te reira takahi o te mahi kupu. I roto i tenei take, mamae kore te wāhanga whakapuaki o te kupu (whakarerekētanga tangi i te tikanga o nga niho, arero, me korokoro), me te kaha ki te whakapuaki i te oro i roto i te taumata pūnaha io pokapū. E te ara, he mea i roto i etahi wāhi i roto i te uho kaka.

Puta ana te mate i roto i ngā tamariki tino hauora. Tuatahi ngaro ratou i te kaha ki te urutau ki te taiao o waho, me kore e taea ka e whakaputa, mārama me arorau faaite ratou whakaaro. Ko te take i roto i te roro o te tamaiti he huringa pathological, whakaatu ana te kite. Te syndrome whakaahuatia i roto i te 1957 e rua pūtaiao - Landau Kleffner. Na reira, o ratou ingoa, me te karanga i tenei mate.

Ko te take o te mate

e kore te tino tautuhi take o Landau-Kleffner syndrome. Ahakoa i roto i ētahi wā, ka taea e ngā tākuta te tautuhi i te take e arahina ki te whanaketanga o te mate. I roto i te wahi tuatahi - ko te whara upoko kati, roro puku, he mate Parasitic meinga e te torongū o te poaka tapeworm - neurocysticercosis. Ko te take i taea e kia, me autoimmune demyelinating mate, i roto i nei i reira ko te whakangaromanga o te mea ma o te pūnaha io - myelin. Tīmata te whanaketanga o te syndrome e taea o mumura i roto i te taiepa oko i te rohe o te roro hoki. ngā takanga maha o mate kua pā ana ki te hē o te matatini romahā punaraungao. I te tahi taime te take hei uho kikokore rawa, hua i roto i te tamaiti whakawhanake i te tuakoi pānga.

Ka taea e te syndrome hei puka fulminant. Ko inanahi haere he kōhungahunga tau-rua, korero, a ko te tamaiti maitai hauora, a inaianei oho ake ia, taea ki te here, me te kupu torutoru. I te tahi taime, te mate kauneke āta, kongakonga āta ngaro tona kaha ki te kōrero ā-waha, me te tika tirohanga o mooni.

He aha e nga tohumate?

mate pūtaiao wahine whakaahuatia i roto i te taipitopito Alexandrov N. Sh Landau-Kleffner syndrome, e ai ta ia, i reira he ano hoki o te plasticity roro pera-ka karanga. Ko te rite ki "whakahou" ki te mahi i pai ratou mahi. I roto i te tukanga o tenei hurihanga, me te whakawhanake i te aphasia rangitahi. Ko te take ki te puka riro, whara me mate meinga e tenei momo o te māuiuitanga e taea te pāngia te whakamahi i Tirohia te rite. I tua atu, kia te "pere tuatahi" hei haurangi hunga haurangi, i puta i te koati. Ko ratou i roto i te 70% o ngā take haere tahi i tīmatanga o te mate.

Huri noa i te hauwhā o te wā katoa, ngaro te manawanui mahi kupu mo te marama. Ko te nuinga o tona mate puta tata hangatonu. I te tahi taime e kore e taea e te tamaiti puaki te reo. nga tūroro hawhe kite disorders whanonga. Kei te tiaki whakaaro whakahaere. Ki te tau, i te haurangi ngaro. whakaora tonu te kanohi ranei o mahi kupu puta hoki te 16 tau.

ino o te kupu

Landau-Kleffner syndrome i roto i te tamaiti haamata ki te mate o te mahi o te reo - ko tetahi o nga tohu matua o te mate tenei. Kei te āhuatanga i te reira na roto i te agnosia-waha māna ranei - waimaero perception, e tiaki i te mahara, me te tūtohutanga. I roto i te take tuatahi, taea ki te korero tika i te manawanui ko te. I roto i te tuarua - e kore e taea e ia i mohio ki reo maheni me ngā oro. I roto i tenei take, he whakautu tonu ki te audiogram oro tātaritanga.

Ko te raruraru matua takoto ïa i roto i te mara o te tūtohutanga tangi. Ahakoa tenei, faaite te syndrome ake i roto i te wā tino o te pūrere hopu reo. Ka taea e purei kupu waimaero hei taumata rerekē o te whīwhiwhi. he nui te kaha i roto i te tahi mau tūroro Aphasia. Ka rite ki te mea kaipūtaiao, kua pāngia take uaua i roto i te 6 tūroro i roto o 77. Ki te mea kua kitea te tamaiti maumaui iti, ka taea e te whakaora i te reira i te pānui, me te tuhituhi pūkenga. raruraru Reo whakakitea e whai ake: confuses te tamaiti reta me tangi he ki te hanga rerenga kōrero, e kore e taea e arorau faaite ratou whakaaro, me te pera i runga i.

haurangi

E kitea haurangi Haumanu i roto i 70-85% o ngā take o riro syndrome Landau-Kleffner. Raruraru i roto i te toru o ngā tamariki haamata ki te ärai haurangi kotahi. Ko tenei - te tīmatanga o te mate. e rave rahi tau kia tangohia e te maha o te tūroro i waenganui i te whawhai me te kupu disorders. Kei te maha haurangi whakaritea i roto i te tau i te 4 ki te 10 tau. He tokomaha taote mea e kore ratou e whai ki te tupu i roto i te ora pakeke. Ko, i muri i te 16 tau o hukihuki kore e tupu.

I roto i te 58% o te tūroro ki te whakaritea haurangi wāhanga. Ratou take tūkino ki te pūtau roro i roto i te wāhi motuhake o tetahi tuakoi kaka. Haurangi puta i roto i te 39% o ngā tamariki, haurangi ki mate o te mahara - i 16%. kitea ētahi tūroro disorders myoclonic pa ana nga uaua o te mata, me te aporo kanohi. I roto i te 12% o tūroro haurangi tuku te i te taumata ira.

disorders whanonga

He āhuatanga hoki tata katoa tūroro ratou. 78% o ngā tamariki taitamariki i raruraru whanonga ki te e pāngia ratou ki Landau-Kleffner syndrome. Aua ra e miharo e te meka e ohorere ka pukuriri ratou pēpi kotahi nonga, me te aroha ngā pakeke. Riri - he tohumate paerewa. Ka taea e te riri e pera kaha, me te haere noa, e kore e taea e te tamaiti utu ki te akakoromaki i reira, kahore hohipera i roto i te whare hinengaro. Etahi, i runga i te anga ke, hoko mawehe koimutu. karo ratou te kamupene o te iwi e mate ana i whakaaro pokepoke manukanuka me.

titiro ētahi tamariki ki te kia tino turi. Etahi hoatu te tātaritanga he - autism. Ko e mo'oni, ko ētahi wā uaua ki te whakatau i te mea te take tūturu o te whanonga ke o te syndrome Landau-Kleffner tākuta. I muri i te katoa, ia me te whanonga autism āhuatanga e ari pērā i te ngoikore ki te hanga kakato arorau o te tono, te tāruarua pokepoke o te taua kīanga, kupu. kukū ngoikore o te aro, me te nui haere mahi kite i roto i te 80% o tūroro. Ahakoa he raruraru whanonga tohu tuarua i roto i te pā ana ki ngā raruraru kupu, kia maere te tahi mau tamariki tauhohe rerekē, huakore, me te rerekē. Hei tauira, ka taea e ratou te whakapau haora tukurua i te taua tohu.

therapy hou

I te tohu tuatahi o te mate, ko te tika ki te timata maimoatanga o Landau-Kleffner syndrome. Te whare hauora, i roto i nei kōrero koe, ka taea ki te whakahere te tahi mau rongoā ki te raruraru:

• akoranga Drug;
• pokanga.

I roto i te take whakamutunga, ka uakina e te manawanui te angaanga, me tango arotahi haurangi. Ki te mahi i tenei, hatepea atu etahi o nga wheua, me o te anga pakeke o te roro. I muri i taua, ko te mea hina o te taote hanga he kokoti. ka tonu ratou ki te aukati i te pakarutanga me horapa o ngātere epileptogenic. Na e hoatu te mater Rura me nga wheua i roto i te wahi. sutured te marutanga te. I muri 6-7 wiki i muri i te pokanga, te tamaiti i te fare ma'i e taea hoki te kāinga. I muri i te pokanga i roto i ngā tamariki ki Landau-Kleffner syndrome whakapai ake kite i roto i te mahi hinengaro, me te hinengaro.

Anti-ärai

e kore te mea tonu whakarato kē whakahaere anake Treatment o Landau-Kleffner syndrome. I te tahi taime e tauturu te reira ki te pehea e o te therapy mate hauora. E whakarato ana fariiraa o anticonvulsants. Kia aroha mai, e kore e hoatu nga wa katoa ratou i te hua pai. Hei tauira, "carbamazepine", "Phenobarbital", "Phenytoin" e whai hua ana maha - e taea te anake aphasia tiki kino. Ka korerotia haurangi i roto i te tūroro ki te rapu utu. Ko rawa pono o tūroro ki hukihuki auau haurangi ngaro mohiotia ranei tenei.

I tua atu, maha tūtohu taote "Nicardipine". E tauturu te reira ki te aukati takere konupūmā. Whai hua ko te whakahaere o te globulin kama rō-uaua. a taua kua whakamatauria makona whai hua tarukino "ethosuximide," "waikawa valproic". Āwhina me benzodiazepines, rawa "Clobazam" me "Midazolam". Ko te tikanga kia whakahaerea e ratou e pâmuhia.

atu raau taero

Landau-Kleffner syndrome, tohu me maimoatanga e whakaahuatia i roto i tenei tuhinga whakarato mo te fariiraa o "Diazepam" i mua i te haere ki te moenga. Tenei raau homai hua pai. i whakaritea therapy te mo te wā o 6 wiki ki te karo tachyphylaxis - pānga whakaiti tere ki te whakamahi toutou o te tarukino. Kua whakaaturia Studies e kia ai taua mea whai hua "levetiracetam". Kei te tino pai te hoki tauturu te reira i roto i te maimoatanga o te tamarikitanga haurangi ngahunga idiopathic, mana hiko haurangi o moe, pehanga o ngaru titi tonu i roto i tona wāhanga puhoi. I tua atu, angitu i roto i te maimoatanga o te aphasia me hukihuki, te tarukino "felbamate." Otira ka whakamahi koe i te mea e taea ki te whakawhanake i te whānuitanga o ngā pānga taha, i roto i nei te tino pinepine tūpono aplastic anemia me ate pōrearea.

corticosteroids

I roto i te maimoatanga o Landau-Kleffner syndrome me te tauturu corticosteroids. "ACTH" ngā pai-kï. whakaiti te reira i te faaiteraa o tohu o riro aphasia hunga haurangi, normalizes te āhua o te kite. therapy wā-roa ki enei taero tūtohutia te nuinga o nga tūroro. Heoi, kia mōhio e kore katoa te mea e tika ana kōwhiringa maimoatanga hormonal.

E mea pinepine tākuta whakahau steroids ki tūroro. Hei tauira, whakahaere "Prednisolone" Kei te pâmuhia runga toru marama. Kei te whakamahia te therapy mō te murunga arataki. He mea nui ki te mau i roto i te ngakau e te whakaiti i roto i te inenga o steroids te maha e pā ana ki hohokitanga tamau o tohu mate. Ko te katoa roanga o te akoranga e taea muri i te ono marama ki te maha o nga tau. Ratau, maimoatanga corticosteroid he rota o ngā pānga taha pērā i taonga taimaha, takawhita, mate huka, awhiowhio wai, hēnga o ōna whatukuhu, korekore, härukiruki syndrome, me te pera i runga i. I tua atu, e kore e tūtohutia ratou mo te tūroro e whai raruraru ki te wātea i te mate, ka rite ki ratou lōmekina atu ia. E contraindicated ano ratou i roto i te iwi ki ngā iroiti necrosis, mate kohi mate ranei whakapataritari nga mahi faufaa o roundworms nei ahua.

Ko nga whakaaro o ngā mātua

hiahiatia tiaki motuhake, me te aro te mo te tamaiti pāngia ki Landau-Kleffner syndrome. Reviews o ngā mātua tohu e hiahia te tamaiti ki te tango i ngā akomanga mō te kaihaumanu reo, Pathologists kupu. E kore e taea e te tākuta whakahoki te mahi kupu. Ko whakaako enei wā te kongakonga ki te manawanui, kawea i roto ia ia te hopoi'a, whakahaere. tautohe ngā mātua e ngā tamariki ki waho pera whakahau te anticonvulsant pai, me te tarukino antiepileptic i huaina "Keppra". tūtohu mātou hoki "Depakine," engari reira pinepine he pānga tōraro i runga i te repe taiaki, na etahi whakawhitinga ki te maimoatanga homeopathic. Kua tuhia hoki maha whaea me matua e te ara pai ki te syndrome - höu pēpi. whiwhi ia pakeke - Kei te āta whakapai ake tona kupu. I tua atu, tau-rere patua me paroxysms.

outlook

Ka rite ki mo nga tākuta, tonu hoki ratou ki te tūhura ki ta te syndrome Landau-Kleffner. tūroro Photo me ratou wāhanga ataata, hītori, mutu tohunga kōtaha kuiti - he wāhanga o te rangahau tenei katoa. Doctors ite e mahi pai i roto i te kaupapa e haere te tamaiti hinengaro, akomanga reo te therapy. Ko tino onge whakaora tonu o ngā mahi ā-waha. Ko te tikanga 2/3 o ngā tamariki i whakaorangia ake ranei iti iho taumaha e whango kupu.