Cov tsos mob thiab kev kho mob ntawm Landau-Kleffner syndrome

Landau-Kleffner syndrome - no mas yuav kis epileptic aphasia nyob rau hauv cov me nyuam. Nws yog yus ua txhaum ntawm kev hais lus muaj nuj nqi. Nyob rau hauv cov ntaub ntawv no, raug kev txom nyem tsis yog tus articulating Cheebtsam ntawm kev hais lus (suab hloov los ntawm txoj kev cov hniav, tus nplaig, thiab lub caj pas), thiab muaj peev xwm articulate lub phoneme nyob rau hauv lub central lub paj hlwb lawm. Los ntawm txoj kev, nws yog nyob rau hauv tej qhov chaw nyob rau hauv lub cerebral cortex.

Tus kab mob no tshwm sim nyob rau hauv kiag li noj qab nyob zoo rau cov me nyuam. Thawj lawv poob rau hauv lub peev xwm hloov mus rau lwm cheeb tsam, thiab ces yuav tsis tau qhia, kom meej meej thiab txawj qhia lawv cov kev xav. Qhov tseeb hais tias nyob rau hauv lub hlwb ntawm tus me nyuam yog pathological kev hloov, qhia tau hais tias lub EEG. Lub syndrome piav nyob rau hauv 1957 los ntawm ob zaum - Landau Kleffner. Yog li ntawd, lawv cov npe thiab hu rau tus kab mob no.

Cov ua ntawm tus kab mob

Ua rau Landau-Kleffner syndrome yog tsis txhais. Txawm tias nyob rau hauv tej rooj plaub, kws kho mob yuav qhia tau tias ib qhov zoo tshaj uas coj mus rau txoj kev loj hlob ntawm tus kab mob. Nyob rau hauv thawj qhov chaw - yog lub kaw lub taub hau raug mob, lub hlwb mob, ib tug cab tus kab mob tshwm sim los ntawm cov kab ntawm nqaij npuas tapeworm - neurocysticercosis. Yog vim li cas yuav tsum tau, thiab autoimmune demyelinating kab mob, nyob rau hauv uas muaj yog kev puas tsuaj ntawm cov dawb teeb meem ntawm poob siab system - myelin. Ua txoj kev loj hlob ntawm lub syndrome kuj muaj peev xwm ntawm o nyob rau hauv cov hlab phab ntsa nyob rau hauv lub cheeb tsam ntawm lub paj hlwb. Ntau occurrences ntawm cov kab mob tau txuam nrog ib tug tsis muaj peev xwm ntawm mitochondrial pa complex. Tej zaum qhov ua ua heev nyias cortex, uas ua rau tus me nyuam tsim lub cuam tshuam hemisphere.

Lub syndrome yuav ua tau fulminant daim ntawv. Qhov ntawd yog nag hmo ib ob-xyoo tus me nyuam taug kev, sib tham, thiab yog txig noj qab nyob zoo tus me nyuam, thiab tam sim no nws wakes li, tsis tau khi, thiab ob peb lo lus. Tej zaum, tus kab mob loj zuj zus maj mam, ib qho kuam me me maj mam loses nws muaj peev xwm mus hais lus kev sib txuas lus thiab txaus xaav ntawm kev muaj tiag.

Yuav ua li cas yog cov tsos mob?

poj niam paub txog tus kab mob tau piav nyob rau hauv kom meej Alexandrov N. Sh Landau-Kleffner syndrome, nws hais tias, muaj kuj yog vim hais tias ntawm lub thiaj li hu ua lub hlwb plasticity. Nws yog raws li yog hais tias "restructured" kom zoo dua ua lawv zog. Nyob rau hauv tus txheej txheem ntawm no transformation thiab muaj ib ntus aphasia. Raws li yog cov ntaub ntawv nrog lub mas yuav kis tau daim ntawv no, kev raug mob thiab cov kab mob tshwm sim los ntawm no hom ntawm pathology yuav kuaj siv EEG. Nyob rau hauv tas li ntawd, lub "thawj tswb" tej zaum yuav ua epileptic qaug dab peg, uas nyob ntawm cov me nyuam yaus. Lawv yog cov nyob rau hauv 70% ntawm cov neeg nrog pib ntawm tus kab mob.

Nyob ib ncig ntawm ib tug peb lub hlis twg ntawm txhua tus neeg mob, tus neeg mob loses hais lus muaj nuj nqi rau ib lub hlis. Tab sis feem ntau ntawm nws tsis tshwm sim zoo instantaneously. Tej zaum ib tug me nyuam muaj peev xwm tsis tso ib suab. Ib nrab ntawm cov neeg mob pom tus cwj pwm mob. Ua hauj lwm xav yog fwm. Nrog lub hnub nyoog, lub qaug dab peg ploj. Tag nrho los yog ib nrab rov qab los ntawm kev hais lus zog tshwm sim rau 16 xyoo.

deterioration ntawm kev hais lus

Landau-Kleffner syndrome nyob rau hauv ib tug me nyuam yuav pib nrog lub poob ntawm muaj nuj nqi ntawm kev hais lus - qhov no yog ib tug ntawm lub ntsiab tsos mob ntawm tus kab mob. Nws yog yus muaj los ntawm cov lus los yog hnov agnosia - impaired xaav, uas khaws cia rau pem thiab rhiab heev. Nyob rau hauv thawj cov ntaub ntawv, tus neeg mob yog tsis txawj hais lus kom zoo. Nyob rau hauv lub thib ob - nws yuav tsis paub txog paub nrov thiab suab. Nyob rau hauv cov ntaub ntawv no, yog ib txwm teb rau diagnostic phonological audiogram.

Lub ntsiab teeb meem lus dag precisely nyob rau hauv lub teb ntawm lub suab rhiab heev. Dua li no, lub syndrome manifests nws tus kheej nyob rau hauv lub tseem ceeb heev lub sij hawm ntawm lus Askiv ntaus ntawv. Tsis hnov hais lus playback yuav ua tau txawv muaj ntau seem ntawm complexity. Aphasia yog heev muaj zog nyob rau hauv ib co neeg mob. Raws li zaum hais, nyuab ntau yog kuaj nyob rau hauv 6 tus neeg mob tawm ntawm 77. Yog hais tias tus me nyuam tau cai me kev ua txhaum, nws yuav cawm tau rau kev nyeem ntawv thiab sau ntawv. Language teeb manifested raws li nram no: tus me nyuam confuses cov tsiaj ntawv thiab cov suab tsis ncaj ncees lawm kom lawv muaj ua ke, yuav tsis txawj qhia lawv cov kev xav thiab hais txog.

qaug dab peg

Soj ntsuam qaug dab peg muaj cai nyob rau hauv 70-85% ntawm cov neeg ntawm mas yuav kis tau Landau-Kleffner syndrome. Kev mob nkees nyob rau hauv ib feem peb ntawm cov me nyuam pib nrog ib tug hluas epileptic qaug dab peg. Qhov no yog - pib ntawm tus kab mob. Ib tug xov tooj ntawm cov neeg mob ntawm tus nres thiab hais lus ntshawv siab tej zaum yuav siv li ob peb lub xyoos. Qaug dab peg yog feem ntau tsau nyob rau hauv lub hnub nyoog los ntawm 4 mus rau 10 xyoo. Muaj ntau cov kws kho mob hais tias lawv tsis muaj tshwm sim nyob rau hauv neeg laus lub neej. Hais tias yog, tom qab 16 xyoo ntawm qaug dab peg tsis tshwm sim.

Nyob rau hauv 58% ntawm cov neeg mob nrog tas ib nrab qaug dab peg. Lawv ua puas mus rau lub hlwb lub hlwb nyob rau hauv ib cheeb tsam ntawm ib cerebral hemisphere. Qaug dab peg tshwm sim nyob rau hauv 39% ntawm cov me nyuam, qaug dab peg nrog rau tsis nco qab - nyob rau ntawm 16%. Ib txhia neeg raug myoclonic mob rau cov nqaij ntshiv ntawm lub ntsej muag thiab lub qhov muag apples. Nyob rau hauv 12% ntawm cov neeg mob qaug dab peg kis ntawm cov kev tshuaj ntsuam genetic theem.

tus cwj pwm mob

Lawv yog cov yam ntxwv rau yuav luag tag nrho cov neeg. 78% ntawm cov me nyuam yaus muaj kev coj cwj pwm muaj teeb meem yog hais tias lawv yog mob Landau-Kleffner syndrome. Cov neeg laus yuav tsum tsis txhob yuav ras los ntawm cov fact tias lawv ib zaug thaj yeeb thiab kheev tus me nyuam mam li nco dheev los ua txhoj puab heev. Npau taws heev heev - ib tug txheej txheem mob. Npau taws yuav ua tau li ntawd, khaus thiab tshwm, tias tus me nyuam tsis muaj nyiaj them mus rau tiv nrog nws tsis muaj tsev kho mob nyob rau hauv ib tug mob hlwb lub tsev kawm ntawv. Lwm tus, on qhov tsis tooj, ua rho dheev. Lawv zam lub tuam txhab ntawm cov neeg kev txom nyem los ntawm kev ntxhov siab vim thiab obsessive xav.

Ib txhia me nyuam zoo li yuav kiag li lag ntseg. Lwm tus muab tso rau lub tsis ncaj ncees lawm mob - tsi txawj has lug. Tseeb, cov kws kho mob no tej zaum kuj tsis yooj yim los mus txiav txim yog dab tsi tiag tiag kom muaj tus coj txawv txawv cwj pwm ntawm ib tug Landau-Kleffner syndrome. Tom qab tag nrho, nws thiab autistic tus cwj pwm yus phenomena xws li lub cev tsis tsim zajlus kom tiav ntawm lub tswv yim, lub obsessive repetition ntawm tib nqe lus, cov lus. Tsis muaj zog concentration ntawm cov xim thiab muaj zog ua si cai nyob rau hauv 80% ntawm cov neeg mob. Txawm tias tus cwj pwm muaj teeb meem yog theem nrab cov tsos mob nyob rau hauv relation mus hais lus teeb meem, ib txhia me nyuam tej zaum yuav xav tsis thoob txawv txawv, tsis thwj thiab bizarre tshua. Piv txwv li, lawv yuav siv sij hawm rov qab ua dua tib piav tes piav taw.

niaj hnub kho

Thaum tus thawj kos npe rau ntawm tus kab mob no yog tsim nyog los pib kev kho mob ntawm Landau-Kleffner syndrome. Lub tsev kho mob, nyob rau hauv uas koj xa, yuav tsum tau muaj ib co kev daws rau qhov teeb meem:

• Drug thaum kawg;
• phais.

Nyob rau hauv lub caij nyoog kawg no cov ntaub ntawv, tus neeg mob qhib lub pob txha taub hau thiab tshem tawm cov epileptic kom pom tseeb. Ua li no, txiav tawm ib co ntawm cov pob txha thiab lub zog plhaub ntawm lub paj hlwb. Tom qab hais tias, lub grey teeb meem ntawm tus kws phais ua ib qho kev incision. Lawv tseem yuav tiv thaiv kom txhob lub phaum thiab kis ntawm epileptogenic pulses. Ces tus Dura mater thiab cov pob txha yog muab tso rau hauv qhov chaw. Cov txhab yog sutured. Tom qab 6-7 lub lis piam tom qab phais, tus me nyuam los ntawm lub tsev kho mob muaj peev xwm rov qab mus tsev. Tom qab muab hlais tawm lawm nyob rau hauv cov me nyuam nrog Landau-Kleffner syndrome pom txoj kev txhim kho nyob rau hauv kev txawj ntse thiab lub hlwb khiav dej num.

Anti-qaug dab peg

Kev kho mob ntawm Landau-Kleffner syndrome yog tsis ib txwm muab tsuas ua hauj lwm variant. Tej zaum nws yuav pab tau tshem ntawm cov kab mob kev kho mob kev kho mob. Nws muab kev txais tos ntawm anticonvulsants. Tu siab, lawv yeej tsis muab ib tug zoo tshwm sim. Piv txwv li, "carbamazepine", "Phenobarbital", "Phenytoin" yog feem ntau tsis zoo - aphasia yuav tsuas tau zuj zus. Qaug dab peg nyob rau hauv cov neeg mob yuav tau rov qab nrog ib tug ua pauj. Qhov no yog tshwj xeeb tshaj yog muaj tseeb ntawm cov neeg mob uas nquag qaug dab peg los yog atypical tsis tuaj kawm ntawv qaug dab peg.

Nyob rau hauv tas li ntawd, cov kws kho mob feem ntau pom zoo kom "Nicardipine". Nws yuav pab tau thaiv calcium raws. Pab tau yog tus thawj coj ntawm tso dej gamma globulin. thiab xws li muaj pov thawj txaus zoo tshuaj "ethosuximide," "valproic acid". Pab thiab benzodiazepines, tshwj xeeb tshaj yog "Clobazam" thiab "Midazolam". Tab sis lawv yuav tsum tau muab intravenously.

lwm yam tshuaj

Landau-Kleffner syndrome, cov tsos mob thiab kev kho mob uas yog tau piav nyob rau hauv no tsab xov xwm muab rau lub txais tos ntawm "Diazepam" ua ntej yuav mus pw. Qhov no tshuaj muab zoo tshwm sim. Therapy yog kws kho rau ib lub sij hawm ntawm 6 lub lis piam kom tsis txhob tachyphylaxis - ceev ceev yuav txo tau cov nyhuv nrog rov siv ntawm cov tshuaj. Kev tshawb fawb pom tau hais tias nws tej zaum yuav pab tau "levetiracetam". Nws kuj zoo heev pab nyob rau hauv cov kev kho mob ntawm thaum yau idiopathic focal qaug dab peg, epileptic hluav taws xob raws li txoj cai ntawm cov pw tsaug zog, tsuj ntawm tas mus li ntev tsis nyob rau hauv nws qeeb theem. Nyob rau hauv tas li ntawd, muaj kev vam meej nyob rau hauv cov kev kho mob ntawm aphasia thiab qaug dab peg, cov tshuaj "felbamate." Tab sis thaum koj siv nws yog ua tau los tsim ib tug ntau yam ntawm cov kev phiv, cov uas lub feem ntau ces yuav tsum aplastic anemia thiab siab kawg.

corticosteroids

Nyob rau hauv cov kev kho mob ntawm Landau-Kleffner syndrome thiab pab corticosteroids. "ACTH" tshwj xeeb zoo-cov pov thawj. Nws thiaj li manifestation ntawm cov tsos mob ntawm nrhiav epileptic aphasia, normalizes lub xeev ntawm cov EEG. Long-term kho nrog cov tshuaj yog pom zoo feem ntau ntawm cov neeg mob. Txawm li cas los, yuav tsum paub hais tias tsis yog txhua leej txhua tus yog tsim hormonal kev kho mob xaiv.

Feem ntau cov kws kho mob muab cov tshuaj steroids rau cov neeg mob. Piv txwv li, "Prednisolone" yog muab intravenously tshaj peb lub hlis. Cov kev kho yog siv rau txoj kev zam txim induction. Nws yog ib qho tseem ceeb los ua tim nyob rau hauv lub siab hais tias tus yuav txo tau nyob rau hauv ntau npaum ntawm steriods kho yog feem ntau txuam nrog rau qhov tsis tshua mob heev ntawm tus kab mob mob. Tag nrho cov duration ntawm lub Hoob no yuav nyob ntev li ntawm rau lub hlis mus rau tsheej xyoo. Incidentally, corticosteroid kev kho mob muaj ib tug ntau ntawm cov kev phiv xws li koj qhov hnyav nce, kub siab, ntshav qab zib, cataracts, raum tsis ua hauj lwm, kev nyuaj siab, manic syndrome thiab thiaj li nyob. Nyob rau hauv tas li ntawd, lawv tsis pom zoo rau cov neeg mob uas muaj teeb meem nrog kev tiv thaiv, raws li lawv ntxiv mluas nws. Lawv kuj txhob nyob rau hauv cov neeg uas muaj aseptic necrosis, mob ntsws los sis cov kab mob uas nws tsos provoked qhov tseem ceeb heev functions ntawm roundworms.

Lub views ntawm cov niam txiv

Tshwj xeeb kev kho mob thiab saib xyuas yuav tsum tau rau ib tug me nyuam mob Landau-Kleffner syndrome. Xyuas ntawm cov niam txiv qhia tau tias ib tug me nyuam yuav tsum siv sij hawm kawm ntawv rau ib tug hais lus therapist, hais lus pathologists. Tus kws kho mob yuav tsis rov qab los hais lus muaj nuj nqi. Tab sis cov kev cobqhia no qhia lub crumbs mus rau lub siab, coj nyob rau hauv nws lub luag hauj lwm, lub koom haum. Cov niam txiv sib ceg tias cov me nyuam txawv teb chaws thiaj li muab ib tug zoo anticonvulsant thiab antiepileptic tshuaj hu ua "Keppra". Peb kuj pom zoo kom "Depakine," tab sis nws feem ntau muaj ib tug tsis zoo feem on tus txiav, thiaj li ib co switching rau homeopathic kev kho mob. Muaj ntau leej niam thiab txiv kuj muab sau tseg tias qhov zoo tshaj plaws txoj kev nrog lub syndrome - maturing tus me nyuam. Nws tau txais cov laus - yog maj txhim kho nws hais lus. Nyob rau hauv tas li ntawd, lub hnub nyoog-khiav tawm tsam thiab paroxysms.

outlook

Raws li rau cov kws kho mob, lawv kuj tseem mus tshawb li cas ib tug Landau-Kleffner syndrome. Photo cov neeg mob nrog rau lawv cov video sijhawm uas nws, keeb kwm, xaus lus tshwj xeeb nqaim profile - tag nrho cov no yog ib feem ntawm cov kev tshawb fawb. Cov kws kho mob pom hais tias txoj kev kho tej hauj lwm zoo dua nyob rau hauv cov kev tshwm sim hais tias tus me nyuam mus kawm hlwb, kev hais lus kawm ntawv. Tag nrho rov qab los ntawm hais lus zog yog tsis tshua muaj heev. Feem ntau 2/3 ntawm cov me nyuam txais kev cawm seej ntau dua los yog tsawg loj heev hais lus impediment.