Iimpawu kunye nonyango Andau-Kleffner syndrome

Andau-Kleffner syndrome - sele ufumene aphasia unesathuthwane ebantwaneni. Lubonakala kukwaphula umsebenzi intetho. Kulo mzekelo, andimvumeli amacandelo sokuthetha intetho (ukuguqulwa isandi esebenzisa amazinyo, ulwimi, nomqala), kunye nokukwazi icacise phoneme kwinqanaba kwinkqubo luvo. Ngendlela, ukuba kwiindawo ezithile apha neuron.

Isifo kwenzeka kubantwana abasempilweni ngokupheleleyo. Okokuqala ukuba balahlekelwe ukuze zikwazi ukumelana yemeko ezisingqongileyo, kwaye ke kuba ezichazayo, ngokucacileyo nangengqiqo baveze iingcamango zabo. Inyaniso yokuba ebuchotsheni le nosana utshintsho lwezidumbu, ibonisa EEG. Syndrome echazwe 1957 zizazinzulu ezimbini - Andau Kleffner. Ngoko ke, amagama abo babiza nesi sifo.

Izinto ezibangela izifo

Oonobangela Andau-Kleffner syndrome akachazwanga ngokupheleleyo. Nangona kwezinye iimeko, oogqirha afumane into ezakhokelela uphuhliso sifo. Kwindawo yokuqala - i ukonzakala entloko closed, ithumba ebuchotsheni, isifo ekumfimfitheni eyabangelwa ezidla yehagu tapeworm - neurocysticercosis. Isizathu ingaba, kunye samathambo demyelinating izifo, apho kukho ngokutshatyalaliswa mbandela emhlophe e-nervous system - myelin. Ivule uphuhliso syndrome nazo ziyakwazi ukudumba eludongeni isitya kummandla ingqondo. elivela ezininzi izifo ziye ezinxulumene kunye ukuswela ezintsonkothileyo ayo zemiphunga. Ngamanye amaxesha isizathu sokuba neuron zibhityile kakhulu, nto leyo umntwana ekuphuhliseni ikhweyitha abachaphazelekayo.

Le syndrome kunokuba ifomu fulminant. Leyo izolo yayisakhasa iminyaka emibini bahamba, sathetha, yaye umntwana uphilile, kwaye ngoku uvuka, akakwazi sokuwubopha, kunye namagama ambalwa. Ngamanye amaxesha, isifo iqhubeka ngokucotha, mixture ngcembe uphelelwa ngamandla ayo unxibelelwano ngomlomo nendlela abayibona eyaneleyo leyinene.

Ziintoni iimpawu?

isifo sesayensi female ichazwe ngokwenkcukacha Alexandrov N. Sh Andau-Kleffner syndrome, uthi, kukho kwakhona ngenxa ekuthiwa-plasticity ebuchotsheni. Kwaba ngathi 'lahlengahlengiswa "ukwenza ngcono imisebenzi yabo. Xa inkqubo yale nkqubo yenguqu kuphuhlisa a aphasia yethutyana. Njengoko kunjalo kunye nefom ezifunyenwe, ukwenzakala kunye nezifo ezibangelwa olu hlobo leengxaki ukuba yintoni na unobangela ngokusebenzisa EEG. Ukongeza, i "intsimbi yokuqala 'ukuze babe sokuwa, leyo Wabonakala kid. Ba ngo-70% yamatyala kunye ekuqaleni kwesi sifo.

Around ikota zonke iimeko, isigulana ulahlekelwa umsebenzi intetho inyanga. Kodwa ke uninzi yokulahlekelwa yayo kwenzeka phantse washona. Ngamanye amaxesha usana akanako ukuzithetha isandi. Isiqingatha izigulane wabona iingxaki zokuziphatha. ukucinga Operational liyagcinwa. Ukuguga, ukugula shwaka. recovery ngokupheleleyo okanye olungaphelelanga imisebenzi yokuthetha kwenzeka iminyaka eli-16.

ngokonakala intetho

Andau-Kleffner syndrome emntwaneni luqala ukulahleka umsebenzi yokuthetha - le yenye iimpawu ezingundoqo zesi sifo. Lubonakala yi Agnosia ngomlomo okanye bezandi - kakuhle imbono, apho kulondolozwe sezingqondweni novakalelo. Kwimeko yokuqala, isigulana akakwazi ukuthetha kakuhle. Lwesibini - akakwazi ukubona ingxolo aqhelekileyo nezandi. Kulo mzekelo, ziimpendulo eqhelekileyo audiogram diagnostic awareness.

Ingxaki ephambili ilele kanye endle njengezinobugcalagcala yesandi. Ngaphandle koku, le sifo esizibonakalisa xesha okunzulu isixhobo ezisisiseko zolwimi. Kakuhle lokudlala intetho kunokuba imigangatho eyahlukeneyo ngobunzima. Aphasia beqine kunene kwezinye izigulane. Njengoko izazinzulu, iimeko nezintsonkothileyo abafunyenwe kwizigulane 6 ngaphandle 77. Ukuba umntwana uye waphawula yanyhashwa ezingephi, ukuba ungagcina izakhono zokufunda nokubhala. ukuphazamiseka Ulwimi obonakala ngolu hlobo lulandelayo: umntwana liyazixakekisa iileta kwaye izandi ephosakeleyo ukwakha izivakalisi, asikwazi ngokusengqiqweni ukuvakalisa iingcinga zabo njalo njalo.

sokuwa

ukuxhuzula zonyango kuvandlakanywa 70-85% amatyala ezifunyenwe Andau-Kleffner syndrome. Isifo a esithathwini siqala yaxhuzula ngisho nakanye. Oku - ekuqaleni kwesi sifo. A Inani lezigulane phakathi kuhlaselwa kunye nentetho ukuphazamiseka kungathatha iminyaka emininzi. Ukuxhuzula zidla sisigxina iminyaka emi-4 ukuya kweli-10. Oogqirha abaninzi bathi azinayo kwenzeka ebomini abadala. Oko kukuthi, emva azenzeki kwiminyaka 16 ukuxhuzula.

Ngowe-58% ezigulane kunye uxhuzula inxenye ezisisigxina. wabo umonakalo iiseli nobuchopho kwindawo ethile omnye ikhweyitha lobuchopho. Uxhuzula-39% abantwana, ukuxhuzula kunye nokulahlekelwa osezingqondweni - kwi-16%. Ezinye izigulane baboniswa ukuphazamiseka myoclonic echaphazela izihlunu kobuso kunye apile kwiliso. Ngowe-12% ezigulane sokuwa usasazwa ugqithiselwa kwinqanaba yemfuza.

izifo zokuziphatha

Ngabo uphawu phantse kuzo zonke izigulana. 78% abantwana abaselula babe neengxaki zokuziphatha xa kwafunyaniswa Andau-Kleffner syndrome. Abantu abadala akufuneki kumangaliswa yokuba usana lwabo kanye zoxolo obathandayo ngesiquphe baba ndlongondlongo. Rage - uphawu eqhelekileyo. Umsindo kunokuba kakhulu kangangokuba kwaye lawuleki, ukuba umntwana akakwazi sikwazi ukumelana nayo ngaphandle esibhedlele kwiziko abagula ngengqondo. Abanye, phezu Kunoko, yiba kungarhoxiswa bulula. Abalekela inkampani yabantu abanengxaki ixhala kunye neengcamango esilawulayo.

Abanye abantwana babonakala kuba zizithulu ngokupheleleyo. Abanye ukubeka uxilongo engalunganga - Autism. Eneneni, oogqirha ngamanye amaxesha kunzima ukufumanisa ukuba yintoni unobangela yokwenene indlela yokuziphatha engaqhelekanga ye Andau-Kleffner syndrome. Ngapha koko, yena kunye nokuziphatha autistic luphawulwa ngezenzeko ezifana nokungakwazi ukwakha ukuphelela ubhalo zesindululo, ukuphindaphindwa izenzo ngamabinzana ezifanayo, amagama. yoxinaniso ezibuthathaka ingqalelo kunye nomsebenzi anda aba-80% yezigulane. Nangona iingxaki zokuziphatha iimpawu zamabanga ngokunxulumene iingxaki zokuthetha, abanye abantwana Basenokothuka reactions ezingaqhelekanga, amanqe, kwaye engaqhelekanga. Umzekelo, ukuba bachithe iiyure abaphinda isenzo efanayo.

unyango lwangoku

Xa uphawu yokuqala sifo kuyimfuneko ukuba ukuqala unyango lwe Andau-Kleffner syndrome. Ikliniki, apho nibhekise, uya kuba nako ukunika ezinye izisombululo le ngxaki:

• course Drug;
• uqhaqho.

Kwimeko yokugqibela, isigulane wavula yaba lukakayi, asuse kujoliswe nabanesathuthwane. Ukuze wenze oku, ndinqumle ezinye amathambo kunye iqokobhe nzima ingqondo. Emva koko, lo mbandela izimvi ugqirha yenza imbobo. Ziya kuqhubeka ukunqanda ukuqhambuka kunye nokunwenwa pulses epileptogenic. Emva koko ke mater Dura kunye namathambo zibekwa endaweni. Inxeba sutured. Emva kweeveki 6-7 emva kotyando, umntwana esibhedlele ukubuyela ekhaya. Emva kotyando kubantwana abane Andau-Kleffner syndrome wabona uphuculo kwimisebenzi zengqondo kunye nasengqondweni.

Anti-sokuwa

Unyango Andau-Kleffner syndrome akusoloko inika lobeko sisebenza kuphela. Maxa wambi kuyanceda ukuba balahle nothomalaliso medical sifo. Inika ulwamkelo anticonvulsants. Ngelishwa, azisoloko ukunika iziphumo ezidibanisayo. Umzekelo, "carbamazepine", "Phenobarbital", "Phenytoin" ayisasebenzi - aphasia unokufumana kuphela kakhulu. Ukuxhuzula kwizigulane ziza kuphindwa nge impindezelo. Oku kuyinyaniso ngokukodwa izigulane uxhuzula rhoqo okanye ukugula engekho Nedlac.

Ukongeza, oogqirha isincomo "Nicardipine". Kuyanceda ukuba ukuvala amajelo calcium. Olunoluncedo ulawulo efakwa gamma globulin. kwaye iye yaphumelela ngokwaneleyo iziyobisi "ethosuximide," "acid valproic". Uncedo kunye benzodiazepines, ingakumbi "Clobazam" yaye "Midazolam". Kodwa kufuneka iphathwe yegazi.

namanye amachiza

Andau-Kleffner syndrome, iimpawu kunye nonyango ezo zichazwe kweli nqaku lichaza ulwamkelo "Diazepam" ngaphambi kokuba uye kulala. Eli chiza kuba neziphumo ezihle. Uthomalaliso ezimiselweyo kwisithuba seeveki 6 ukuphepha tachyphylaxis - isiphumo nokwehliswa ngokukhawuleza kunye nokusetyenziswa ngokuphindaphindiweyo beziyobisi. Uphononongo lubonise ukuba kusenokuba luncedo "levetiracetam". Kwakhona kunceda kakuhle kakhulu unyango yobuntwana ukuxhuzula focal idiopathic, ubume zombane unesathuthwane ebuthongweni, kwezithandani kwamaza spike eqhubekayo kwisigaba yakhe kade. Ukongeza, nempumelelo unyango lwe aphasia nokuxutha, ichiza "felbamate." Kodwa xa usebenzisa kunokwenzeka ukuphuhlisa iintlobo ziziphumo ebezingalindelekanga, phakathi apho wadibana qho aplastic anemia zesibindi kwekhono.

corticosteroids

Xa unyango lwe Andau-Kleffner syndrome kunye noncedo corticosteroids. "ACTH" ingakumbi kakuhle aqinisekisiweyo. Oku kunciphisa ngokubonakala iimpawu ezifunyenwe aphasia unesathuthwane, normalizes imeko EEG. Unyango Long-term nala machiza kucetyiswa ezininzi izigulane. Nangona kunjalo, qaphela ukuba wonke umntu ukhetho efanelekileyo hormone unyango.

Ngokuqhelekileyo oogqirha amisele steroids kwizigulane. Umzekelo, "Prednisolone" ilungiselelwayo yegazi phezu iinyanga ezintathu. Uthomalaliso esetyenziselwa kuxolelwe induction. Kubalulekile ukuba kukhunjulwe ukuba ukwehla kwi idosi steroid unxulunyaniswa ukwenzeka rhoqo iimpawu zesifo. Ubude lilonke kwekhosi unokuthatha ukusuka kwiinyanga ezintandathu ukuya kwiminyaka emininzi. Phela, unyango corticosteroid wehlelwa ziziphumo ebezingalindelekanga ezifana ukutyeba, lwegazi, isifo seswekile, cataract, ukusilela kwezintso, ukudakumba, manic syndrome njalo njalo. Ukongeza, ukuba akukhuthazwa ukuba izigulane abanengxaki sifo, njengoko zisidimaze ngakumbi kuye. Kananjalo contraindicated kubantu necrosis aseptic, isifo sephepha okanye izifo imbonakalo ukumqumbisa imisebenzi ebalulekileyo ziintshulube ogama.

Iimbono abazali

yinkathalelo ebalulekileyo kunye ingqalelo kufuneka ukuba umntwana une Andau-Kleffner syndrome. Izimvo abazali zibonisa ukuba umntwana kufuneka athathe neeklasi owayendifundisa ukuthetha, pathologists ntetho. Ugqirha akakwazi ukubuyisela umsebenzi intetho. Kodwa ezi seshoni bafundisa kwiimvuthuluka ukuya unyamezelo, bazise uxanduva kuye, umbutho. Abazali athi abantwana phesheya ukuze amisele i anticonvulsant elungileyo kunye neziyobisi antiepileptic ebizwa ngokuba "Keppra". Siyaleza kwakhona "Depakine," kodwa ke kunefuthe elibi injeke, ngoko abathile utshintshe nje unyango ngamayeza. Oomama abaninzi nootata nazo ziye zaphawula ukuba ndlela ilungileyo kunye syndrome - nokukhulisa umntwana. Igula nangaphezulu - kuxhaphaka ekuphuculeni intetho yakhe. Ukongeza, ubudala-run ukuhlaselwa kunye paroxysms.

Outlook

Ke ukuba oogqirha, nabo kuqhubeka ukuphonononga into Andau-Kleffner syndrome. izigulane Photo yevidiyo iziqephu zabo, imbali, waqukumbela iingcali iprofayile emxinwa - konke oku yinxalenye yophando. Oogqirha waphawula ukuba unyango lusebenza ngcono kwimeko apho umntwana uya Unyango, iiklasi intetho. recovery ngokupheleleyo imisebenzi ngamazwi kahle inqabile. Ngokuqhelekileyo 2/3 abantwana wabasindisa ngaphezulu okanye ngaphantsi kakhulu sithintelo intetho.