Izimpawu kanye nokwelashwa Landau-Kleffner syndrome

Landau-Kleffner syndrome - lutholwa aphasia sokuwa ezinganeni. Lubonakala ukwephulwa umsebenzi yokukhuluma. Kulokhu, ahlupheke hhayi izingxenye Ukubhekisisa yokukhuluma (ukuguqulwa umsindo esebenzisa amazinyo, ulimi, nomphimbo), kanye nekhono nokuphimisela phoneme ku sezinzwa uhlelo ezingeni. By the way, kuba ezindaweni ezithile e cortex cerebral.

Lesi sifo kwenzeka ezinganeni engumqemane. Baqale balahlekelwe ikhono ukuzivumelanisa imvelo bangaphandle, bese akukwazi bayaxoxa, ngendlela ecacile nenengqondo baveze abakucabangayo. Iqiniso lokuthi e ubuchopho ingane izinguquko sokugembula, ikhombisa Bheka. Lesi sifo ezichazwe 1957 ososayensi ababili - Landau Kleffner. Ngakho-ke, amagama abo bese bekhala lesi sifo.

Izimbangela isifo

Izimbangela Landau-Kleffner syndrome akachaziwe ngokugcwele. Nakuba kwezinye izimo, odokotela bangathola ukuthi ukukhomba isici kwaholela ekwakhekeni lesi sifo. Endaweni yokuqala - iyona avaliwe ukulimala ekhanda, ebuchosheni, isifo yisinanakazana kubangelwa izibungu yengulube tapeworm - neurocysticercosis. Isizathu kungaba, futhi sokuhlasela kwamasosha omzimba demyelinating izifo, lapho kukhona kubhujiswa lesi daba ezimhlophe isimiso sezinzwa - myelin. Kushiya ukuthuthukiswa yalesi sifo futhi uyakwazi ukuvuvukala odongeni uyisitsha esifundeni zobuchopho. izenzeko Multiple izifo ziye zahlobana nokuntuleka mitochondrial eziyinkimbinkimbi zokuphefumula. Ngezinye izikhathi imbangela babe cortex kakhulu mncane, okuholela yomntwana osakhula Nenkabazwe abathintekayo.

Lesi sifo kungaba ifomu fulminant. Lokho izolo encane eneminyaka emibili wahamba, wakhuluma, futhi kwaba ingane ephelele, futhi manje yena uyavuka, abakwazi ukuhlanganisa uxhumano nomsebenzisi, futhi amagama ambalwa. Ngezinye izikhathi, lesi sifo sanda kancane kancane, imvuthuluka kancane kancane ilahlekelwa amandla abo okuphila umlilo tekuchumana kanye nombono ezanele engokoqobo.

Ziyini izimpawu?

zesifazane usosayensi isifo wachaza ngokuningiliziwe Alexandrov N. Sh Landau-Kleffner syndrome, uthi, kukhona futhi ngenxa okuthiwa ubuchopho plasticity. Kwaba sengathi "kabusha" ukwenza kangcono imisebenzi yabo. Ngo inqubo lolu shintsho futhi eba aphasia yesikhashana. Njengoba kunjalo nge kuluthola ifomu, ukulimala kanye izifo ezibangwa lolu hlobo zokugula Kungahlonzwa ngomshini usebenzisa Bheka. Ngaphezu kwalokho, "insimbi yokuqala" bangase babe zesifo sokuwa, eyabonakala ngezinye kid. Ziyakwazi 70% amacala ephelezelwa ukuqala lesi sifo.

Duzane ingxenye yesine yayo yonke izimo, isiguli ilahlekelwa umsebenzi yokukhuluma ngoba ngenyanga. Kodwa iningi ukulahlekelwa kwayo kwenzeka cishe ngokuphazima kweso. Ngezinye izikhathi ingane olakho ukukukhuluma umsindo. Isigamu iziguli ukubonwa ezibangela ukuziphatha okuyinqaba. ukucabanga Operational ugciniwe. Njengoba bekhula, ukuquleka zinyamalale. yokutakula ngokugcwele noma kancane yokukhuluma imisebenzi zenzeka iminyaka engu-16.

Ukuwohloka inkulumo

Landau-Kleffner sifo ngo ingane iqala ukulahlekelwa umsebenzi yokukhuluma - lokhu ingenye izimpawu eziyinhloko zesifo. It libhekene ngamazwi noma sokuzwa agnosia - kahle bezibona, okuyinto ukulondoloza unembeza lobukhali obungcono. Esimweni sokuqala, isiguli iyisimungulu kahle. In the yesibili - akakwazi ukubona imisindo ajwayelekile nemisindo. Kulokhu, kukhona izimpendulo evamile zokuxilonga audiogram Imphimiso.

Inkinga eyinhloko itholakala ngokunembile emkhakheni ukuzwela umsindo. Naphezu kwakho konke lokhu, lesi sifo kubonakala esikhathini esibucayi idivayisi ukuzuzwa kwelimi. Kahle inkulumo ukudlala kungaba amazinga ehlukene obulukhuni. Aphasia kunamandla kakhulu kwezinye iziguli. Njengoba ososayensi bathi, izimo eziyinkimbinkimbi uxilongwe ezigulini 6 aphume 77. Uma ingane uzigcinile ukwephula ezincane, kungaba ugcine kwemakhono eKufundza neKubhala. thuthuva Ulimi wabonakaliswa kanje: ingane ephambanisa izinhlamvu umsindo okungalungile ukwakha imisho, awukwazi ngokunengqondo baveze abakucabangayo nokunye.

isithuthwane

ukuquleka Clinical kuyagcinwa e 70-85% of amacala atholwe Landau-Kleffner syndrome. Nokugula endaweni yesithathu izingane iqala kokuquleka ngisho nakanye. Lena - ekuqaleni kwalesi sifo. Iziguli phakathi kuhlasela futhi inkulumo iziyaluyalu kungase kuthathe iminyaka eminingana. Ukuquleka zivame fixed kweminyaka emibili nengu-4 kuya kwezingu-10. odokotela abaningi bathi akudingeki ukuba ezenzeka ekuphileni omdala. Okusho ukuthi, emva engu-16 nokuquleka azisenzeki.

Ngo-58% eziguli nge fixed nokuquleka esinqunyiwe. izimbangela zabo kungalimaza ukuba amangqamuzana obuchopho endaweni ethile eyodwa Nenkabazwe-cerebral palsy. Ukuquleka kwenzeka e-39% izingane, ukuquleka nge nokulahlekelwa ukwazi - at 16%. Ezinye iziguli wadalula iziyaluyalu myoclonic ezithinta imisipha ubuso kanye apula iso. Ngo-12% eziguli isithuthwane idluliselwa ezakhini zofuzo.

ezibangela ukuziphatha okuyinqaba

Ziyakwazi sici for cishe zonke iziguli. 78% izingane ezincane nezinkinga zokuziphatha uma uxilongwe Landau-Kleffner syndrome. Abadala akufanele kusimangaze yokuthi ingane yabo kanye elinokuthula futhi obathandayo ngokuzumayo yaba nolaka. Rage - uphawu ejwayelekile. Ulaka kungaba kukhulu kangangokuba ngokweqile futhi zingalawuleki, ukuthi ingane abanakukwazi naso ngokuphumelelayo ngaphandle esibhedlela esikhungweni sabagula ngengqondo. Abanye, kunalokho, baba kuhoxiswe esithubeni nje. They xwaya inkampani zabantu abaphethwe ekukhathazekeni nemicabango esilawulayo.

Ezinye zezingane kubukeka sengathi ukuba yisithulu ngokuphelele. Abanye wafaka le uphethwe okungalungile - Autism. Ngempela, odokotela ngezinye izikhathi kunzima ukunquma ukuthi iyini imbangela yangempela ukuziphatha ongaziwa we Landau-Kleffner syndrome. Phela, wayehawukela nokuziphatha autistic libhekene ezinjengokungcoliswa ukungakwazi ukwakha ukuphelela okunengqondo sesiphakamiso, ukuphindaphindwa esilawulayo imishwana esifanayo, amazwi. okuhlushwa engaqinile ukunakwa umsebenzi kunyuke ebonwe 80% eziguli. Nakuba izinkinga zokuziphatha kukhona izimpawu yesibili maqondana nezinkinga inkulumo, ezinye izingane kungase kukumangaze ukusabela engavamile, akunangqondo futhi yinqaba. Ngokwesibonelo, bachithe amahora ngokuphinda isenzo esifanayo.

yokwelapha yesimanje

Lapho kuvela izimpawu zokuqala zesifo kuyadingeka ukuze uqale ekwelapheni Landau-Kleffner syndrome. Umtholampilo, lapho ubheke, uzokwazi ukunikela abanye izixazululo inkinga:

• Yiqiniso izidakamizwa;
• ukuhlinzwa.

Kulo mBhalo, isiguli wavula kogebhezi ukususa focus sokuwa. Ukuze wenze lokhu, nginqume ezinye amathambo igobolondo kanzima zobuchopho. Ngemva kwalokho, ompunga endabeni udokotela ohlinzayo kwenza imbobo. Bayoqhubeka ukuvimbela ukugqashuka futhi ukusabalala pulses epileptogenic. Khona-ke Mater Dura namathambo ifakwa endaweni. Isilonda sutured. Ngemva kwamasonto 6-7 emva kokuhlinzwa, ingane esibhedlela ungabuyela ekhaya. Ngemva kokuhlinzwa nezingane Landau-Kleffner syndrome ukubonwa ubungcono imisebenzi bokusungula nezingokwengqondo.

Anti-kokuquleka

Ukwelashwa nokuvikelwa Landau-Kleffner syndrome akuyona njalo ihlinzeka okufana sisebenza kuphela. Ngezinye izikhathi kuyasiza ukuqeda yokwelapha yezokwelapha isifo. Linikeza yokwamukela anticonvulsants. Ngeshwa, abayivezi ngaso sonke isikhathi ukunikeza umphumela omuhle. Ngokwesibonelo, "carbamazepine", "Phenobarbital", "Phenytoin" ngokuvamile ayizwakali - aphasia kungaba ibhebhetheke. Ukuquleka ezigulini kuzophindwa nge impindiselo. Lokhu kuyiqiniso ikakhulukazi iziguli nokuquleka njalo noma atypical ukungabi nokuquleka.

Ngaphezu kwalokho, odokotela bavame ukutusa "Nicardipine". Kuyasiza ukuvimba iziteshi calcium. Ewusizo nokuphathwa emthanjeni-gamma globulin. futhi enjalo iye yafakazela ngokwanele ngempumelelo izidakamizwa "ethosuximide," "valproic acid". Usizo benzodiazepines, ikakhulukazi "Clobazam" futhi "Midazolam". Kodwa kumele iqondiswa ngemithambo yegazi.

nezinye izidakamizwa

Landau-Kleffner syndrome, izimpawu kanye zokwelapha ezichazwe kulesi sihloko sinikeza yokwamukela "Diazepam" ngaphambi kokulala. Lesi sidakamizwa linikeza imiphumela emihle. Ukwelashwa ezinqunyiwe isikhathi esingamasonto 6 ukugwema tachyphylaxis - okusheshayo ukunciphisa umphumela nge Ukulisebenzisa ngokuphindaphindiwe igama izidakamizwa. Ucwaningo luye lwabonisa ukuthi kungase kube usizo "levetiracetam". Iphinde isize ekwelapheni ebuntwaneni idiopathic nokuquleka ebalulekile, nabanesithuthwane isimo kagesi ubuthongo, ukucindezelwa of okuqhubekayo spike amagagasi esigabeni sakhe kancane kahle. Ngaphezu kwalokho, yimpumelelo ekwelapheni aphasia kanye nokuquleka, lesi sidakamizwa "felbamate." Kodwa uma usebenzisa kungenzeka ukuthuthukisa ezihlukahlukene side effects, phakathi okuyinto evame kakhulu ihlangabezane aplastic anaemia kanye isibindi ukungasebenzi.

corticosteroids

Ekwelapheni Landau-Kleffner syndrome ne usizo corticosteroids. "ACTH" ikakhulukazi kahle kungase kutholakale. Kunciphisa ukubonakaliswa izimpawu atholwe aphasia nabanesithuthwane, normalizes isimo Bheka. ukwelashwa eside nalezi izidakamizwa Kunconywa iningi iziguli. Nokho, qaphela ukuthi akuwona wonke umuntu ofanele hormone ukwelashwa okukhethwa kukho.

Ngokuvamile odokotela linqume steroid iziguli. Ngokwesibonelo, "Prednisolone" iqondiswa ngemithambo yegazi phezu izinyanga ezintathu. Therapy isetshenziselwa ukuthethelelwa lokungeniswa. Kubalulekile ukukhumbula ukuthi ukuncipha umthamo steroid ngokuvamile lihlotshaniswa Ukuphinda njalo izimpawu isifo. Ubude obuphelele inkambo ingahlala ngemva kwezinyanga eziyisithupha kuya eminyakeni eminingana. Phela, ukwelashwa ngama-corticosteroid has a lot of komzimba okufana kwesisindo, umfutho ophakeme wegazi, isifo sikashukela, ale nkinga, ukungasebenzi kwezinso, ukucindezeleka, ukwenama syndrome nokunye. Ngaphezu kwalokho, kungukuthi Kunconywa ezigulini unezinkinga amasosha omzimba, njengoba eminye sicindezeleke kuye. Babuye contraindicated kubantu abane necrosis aseptic, isifo sofuba, noma izifo osimo sokucasuka imisebenzi esibalulekile roundworms.

Imibono abazali

ukunakekelwa Special iyadingeka ukuze ingane ukuthi nginesifo Landau-Kleffner syndrome. Izibuyekezo abazali zibonisa ukuthi ingane udinga ukuthatha amakilasi nodokotela osiza abanezinkinga zokukhuluma, inkulumo pathologists. Udokotela ayikwazi ukubuyisela umsebenzi yokukhuluma. Kodwa kulezi zifundo ukufundisa imvuthu ukuze ukuphikelela, ukuletha kuye umthwalo, inhlangano. Abazali bangase baphikise ngokuthi izingane phesheya ngakho linqume anticonvulsant ezinhle futhi izidakamizwa antiepileptic ngokuthi "Keppra". Siphinde ukutusa "Depakine," kodwa ngokuvamile kuba nomthelela omubi kwamanyikwe, ngakho abanye usuke ekwelashweni homeopathic. Obaba nomama abaningi futhi baye baphawula ukuthi indlela engcono kakhulu sifo - ukukhula ingane. Ubulwelwe amadala - kancane kancane ngcono ngenkulumo yakhe. Ngaphezu kwalokho, ubudala ukusebenzisa ukuhlaselwa futhi paroxysms.

ukuba nombono ongafani ngeke

Ngokuqondene odokotela, nabo qhubeka nokuhlola ukuthi Landau-Kleffner syndrome. iziguli Photo ngevidiyo iziqephu, umlando wabo, waphetha Ochwepheshe iphrofayela ewumngcingo - konke lokhu kuyingxenye zocwaningo. Odokotela waphawula ukuthi imishanguzo isebenza kangcono uma kwenzeka ukuthi ingane iyaya kwengqondo, inkulumo amakilasi. babuyele ngokugcwele imisebenzi ngamazwi oluphakeme ezingavamile. Ngokuvamile 2/3 izingane ukulondolozwa ucishe sina ukungakwazi ukukhuluma kahle.