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Ciwon Landau-Kleffner

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Ciwon Landau-Kleffner
Description (en) Fassara
Iri childhood electroclinical syndrome (en) Fassara
cuta
Specialty (en) Fassara neurology (en) Fassara
psychiatry (en) Fassara
Genetic association (en) Fassara GRIN2A (en) Fassara
Suna saboda William Landau (en) Fassara da Frank Kleffner (en) Fassara
Identifier (en) Fassara
ICD-10-CM G40.8
ICD-10 F80.3
ICD-9 345.8
OMIM 245570
DiseasesDB 31407
MeSH D018887
Disease Ontology ID DOID:2538

Landau-Kleffner syndrome (LKS) - wanda kuma ake kira cuta ce mai ban sha'awa tare da samun aphasia, samun aphasia epileptic ko aphasia tare da rikicewar convulsive - cuta ce ta ƙuruciya mai ban shaʼawa.

An sanya masa suna ne bayan William Landau da Frank Kleffner, waɗanda suka bayyana shi a cikin 1957 tare da gano yara shida.

Alamomi da alamomi

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Cutar Landau-Kleffner tana nunawa ta hanyar ci gaba na kwatsam ko sannu a hankali na aphasia (rashin iya fahimta ko bayyana harshe) da kuma electroencephalogram (EEG). LKS yana shafar sassan kwakwalwa waɗanda ke sarrafa fahimta da magana (Yankin Broca da Yankin Wernicke). Cutar yawanci tana faruwa ne a cikin yara tsakanin shekaru 3 zuwa 7. Akwai rinjaye na namiji a cikin ganewar asali na cutar (rashin 1.7:1, maza zuwa mata).

Yawanci, yara masu LKS suna bunkasa yadda ya kamata, amma sai su rasa ƙwarewar harshe. Duk da yake mutane da yawa da abin ya shafa suna da cututtukan asibiti, wasu kawai suna da cututtuka na lantarki, gami da yanayin cututtukani na bacci (ESES). Alamar farko ta matsalar harshe yawanci ana jin agnosia ce ta magana. Ana nuna wannan a cikin marasa lafiya ta hanyoyi da yawa ciki har da rashin iya gane sautin da aka saba da shi da kuma raunin ikon yin amfani da sauti. Bugu da kari, harshe mai karɓa sau da yawa yana da rauni sosai, duk da haka a wasu marasa lafiya, raunin harshe mai bayyanawa shine mafi zurfi. A cikin binciken da aka yi na shari'o'i 77 na cutar Landau-Kleffner, an gano 6 suna da irin wannan aphasia. Saboda wannan ciwon ya bayyana a lokacin irin wannan mawuyacin lokaci na samun harshe a rayuwar yaro, samar da magana na iya shafar kamar yadda fahimtar harshe ke shafar.[1] Farawar LKS yawanci tsakanin watanni 18 zuwa shekaru 13, mafi yawan lokacin fitowa yana tsakanin shekaru 3 zuwa 7.

Gabaɗaya, bayyanar cutar ta farko tana da alaƙa da rashin farfadowar harshe, da kuma bayyanar fashewar dare wanda ke ɗaukar fiye da watanni 36.[1] LKS tana da bambance-bambance masu yawa na alamomi kuma ba ta da daidaito a cikin ka'idojin bincike tsakanin shari'o'i, kuma yawancin binciken ba su haɗa da bin diddigin marasa lafiya ba, don haka ba a san wasu alaƙa tsakanin alamomi da warkewa ba.[2]

Rashin lalacewar harshe a cikin marasa lafiya yawanci yana faruwa a cikin makonni ko watanni. Koyaya, an kuma bayar da rahoton farawar yanayin da kuma fashewar fashewa.

Cutar, musamman a cikin dare, alama ce mai nauyi na LKS. Yaduwar fashewar asibiti a cikin aphasia na farfajiya (LKS) shine 70-85%. A cikin kashi ɗaya bisa uku na marasa lafiya, an rubuta wani abu guda ɗaya na fashewa. Cutar yawanci tana bayyana tsakanin shekaru 4 zuwa 10 kuma ta ɓace kafin ta girma (kimanin shekaru 15). [1]

Sau da yawa, rikice-rikice na halayyar mutum da na jijiyoyin jiki suna tare da ci gaban LKS. Ana ganin batutuwan halayyar a cikin kashi 78% na dukkan lokuta. Ana lura da yawan aiki da raguwar kulawa a cikin kusan 80% na marasa lafiya da kuma fushi, tashin hankali, da damuwa. Wadannan halaye ana daukar su a matsayin na biyu ga raunin harshe a cikin LKS. Rashin ƙwaƙwalwar ajiya na ɗan gajeren lokaci alama ce da aka rubuta a cikin lokuta masu tsawo na kamuwa da cututtukan farfajiya.[1]

Dalilin da ya sa

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Yawancin lokuta na LKS ba su da sanannen dalilin. Lokaci-lokaci, ana iya haifar da yanayin na biyu ga wasu ganewar asali, kamar ƙananan kumburi na kwakwalwa, raunin rufe kai, Neurocysticercosis, da Cutar Demyelinating. Tsakanin Tsakanin jijiyoyin jijiyoyi na iya haɗawa da wannan yanayin.

Binciken ganewa

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Cutar na iya zama da wahala a gano ta kuma ana iya kuskuren gane ta a matsayin jin" data-linkid="68" href="./Autism" id="mwNw" rel="mw:WikiLink" title="Autism">autism, rikicewar ci gaba, raunin ji, nakasa ta ilmantarwa, rikice-rikicen ji / magana, rikice na Rashin kulawa, nakasa ta hankali, schizophrenia na yara, ko matsalolin motsin rai / halayyar. Gwajin EEG (electroencephalogram) yana da mahimmanci ga ganewar asali. Yawancin marasa lafiya da ke nuna LKS za su nuna aikin kwakwalwa mara kyau a bangarorin dama da hagu na kwakwalwa; ana nuna wannan akai-akai yayin bacci.[2] Kodayake karatun EEG mara kyau ya zama ruwan dare a cikin marasa lafiya na LKS, ba a gano dangantaka tsakanin matsalolin EEG da kasancewar da tsananin matsalolin harshe ba. A lokuta da yawa duk da haka, abnormalities a cikin gwajin EEG ya riga ya riga ya lalacewar harshe kuma ci gaba a cikin bin diddigin EEG ya riga inganta harshe (wannan yana faruwa a kusan rabin dukkan yaran da abin ya shafa). Abubuwa da yawa suna hana amincin bayanan EEG: rashi na jijiyoyi ba ya bin manyan canje-canjen EEG a cikin lokaci.[1]

Hanyar da ta fi tasiri don tabbatar da LKS ita ce ta hanyar samun EEGs na barci na dare, gami da EEGs a duk matakai na barci. Za'a iya kawar da yanayi da yawa kamar demyelination da kumburi na kwakwalwa ta hanyar amfani da magnetic resonance imaging (MRI). A cikin LKS, binciken fluorodeoxyglucose (FDG) da positron emission tomography (PET) na iya nuna raguwar metabolism a cikin ɗaya ko duka lobes na lokaci - an ga hypermetabolism a cikin marasa lafiya tare da samun aphasia epileptic.[1]

Yawancin lokuta na LKS ba su da sanannen dalilin. Lokaci-lokaci, ana iya haifar da yanayin na biyu ga wasu ganewar asali kamar ƙananan kumburi na kwakwalwa, rauni na rufe kai, Neurocysticercosis, da cututtukan demyelinating. Tsakanin Tsakanin jijiyoyin jijiyoyi na iya haɗawa da wannan yanayin.[1]

Binciken bambance-bambance

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Teburin da ke ƙasa yana nuna bayyanar cututtuka mai yawa da bambanci na bayyanar cututtukan cututtukani tare da Cognitive da Halin Halin Halitta: [1]

Binciken ganewa Rashin lalacewa Tsarin EEG
Rashin farfajiya na autistic Harshen bayyanawa, RL, S, magana da magana ba tare da magana ba Tsakanin tsakiya
Rashin koma baya na Autistic Harshen bayyanawa, RL, S, magana da magana ba tare da magana ba Al'ada
Rashin farfajiya da aka samu RL, mai yiwuwa halayyar Hagu ko dama na lokaci ko na parietal, mai yiwuwa ESES
Samun bayyanar farfajiya Harshen bayyanawa, oromotor apraxia Tsakanin tsakiya
(Yana da Littafi Mai Tsarki) Harshe mai bayyanawa, RL, mai yiwuwa halayyar ESES
Ci gaban dysphasia (ciwon bayyanar harshe na ci gaba) A'a; rashin samun harshe mai bayyanawa Sikeli na lokaci ko na parietal
Rashin farfajiya Harshen bayyanawa, RL, S, sadarwa ta baki da ba ta magana ba, mai yiwuwa halayyar ESES
Tsinkaya Harshen bayyanawa, babu maganganun magana da jin Spike-and-wave discharges a hagu temporocentral da frontal yankuna [3]

Lura: EEG = electroencephalographic; ESES = yanayin lantarki na bacci; RL = harshe mai karɓa; S = zamantakewa

  • Ci gaba da hauhawa da raƙuman barci mai jinkiri (> 85% na barci mai jinkirin).

Magani ga LKS yawanci ya kunshi magunguna, kamar su anticonvulsants [4] da corticosteroids [5] (kamar prednisone), [6] da kuma maganin magana, wanda ya kamata a fara da wuri. Wasu marasa lafiya sun inganta tare da amfani da corticosteroids ko hormone na adrenocorticotropin (ACTH) wanda ke jagorantar bincike don gaskata cewa kumburi da vasospasm na iya taka rawa a wasu lokuta na aphasia na epileptic.[1]

Wani zaɓi na magani mai rikitarwa ya haɗa da dabarar tiyata da ake kira Multiple subpial transection [7] wanda ake yin yanka da yawa ta hanyar cortex na ɓangaren kwakwalwa da ya shafa a ƙarƙashin Pia mater, yana yanke sassan axonal a cikin fararen abu. Ana yanka cortex a layi daya zuwa tsakiyar lokaci gyrus da yankin perisylvian don rage yaduwar aikin epileptiform ba tare da haifar da dysfunction cortical ba. Akwai binciken da Morrell et al. suka yi wanda aka bayar da rahoton sakamako ga marasa lafiya 14 da ke fama da cututtukan farfajiya waɗanda suka sha wahala da yawa. Bakwai daga cikin marasa lafiya goma sha huɗu sun dawo da magana mai dacewa da shekaru kuma ba sa buƙatar maganin magana. Wani 4 daga cikin 14 ya nuna ingantaccen magana da kuma fahimtar umarnin da aka ba da baki, amma har yanzu suna buƙatar maganin magana. Marasa lafiya goma sha ɗaya suna da matsalar harshe na shekaru biyu ko fiye.[8] Wani binciken da Sawhney et al. suka yi ya ba da rahoton ci gaba a cikin dukkan marasa lafiya uku da suka kamu da cutar farfajiya wadanda suka sha wahala iri ɗaya.[9]

Asibitoci daban-daban suna dauke da shirye-shiryen da aka tsara don magance yanayi kamar LKS kamar Asibitin Yara na Boston da Shirin Sadarwa na Ƙara. An san shi a duniya saboda aikinsa tare da yara ko manya waɗanda ba sa magana ko kuma suna da rauni sosai. Yawanci, ƙungiyar kulawa ga yara masu LKS ta ƙunshi likitan jijiyoyi, likitan ƙwaƙwalwa, da likitan magana ko likitan ji. Wasu yara da ke da matsalolin halayyar na iya buƙatar ganin masanin ilimin halayyar yara da masanin ilimin likitan halayyar mutum. Magungunan magana suna farawa nan da nan a lokacin ganewar asali tare da maganin likita wanda zai iya haɗawa da steroids da magungunan rigakafi ko magungunan da ke hana fashewa.

Ilimi na haƙuri ya kuma zama mai taimako wajen magance LKS. Koyar da su yaren kurame hanya ce mai taimako ta sadarwa kuma idan yaron ya iya karatu da rubutu kafin farawar LKS, wannan ma yana da matukar taimako.

Hasashen da aka yi

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Hasashen yara masu cutar LKS ya bambanta. Wasu yara da abin ya shafa na iya samun matsala mai tsanani na harshe na dindindin, yayin da wasu na iya dawo da yawancin iyawarsu ta harshe (ko da yake yana iya ɗaukar watanni ko shekaru). A wasu lokuta, raguwa da sake dawowa na iya faruwa. Ana inganta hangen nesa lokacin da farkon cutar ya kasance bayan shekaru 6 kuma lokacin da aka fara maganin magana da wuri. Cutar ta ɓace gabaɗaya ta hanyar girma. Rashin lafiya na ɗan gajeren lokaci ba sabon abu ba ne a cikin LKS amma suna haifar da matsaloli wajen kimanta martani na mai haƙuri ga hanyoyin warkewa daban-daban.

Tebur mai zuwa yana nuna Binciken Tsawon Lokaci na Aphasia da aka samu a cikin nazarin kayan aiki daban-daban:[1]

Nazarin Adadin Marasa Lafiya Yana nufin bin diddigin, y Adadin Marasa Lafiya da Matsalolin Harshe na al'ada ko na Ƙananan
Soprano et al. (1994) 12 8 3
Mantovani da Landau (1980) 9 22 6
Paquier (1992) 6 8.1 3
Rossi (1999) 11 9.7 2
Robinson et al. (2001) 18 5.6 3
Duran et al. (2009) 7 9.5 1
Jimillar 63 18 (28.6%)

An bayar da rahoton ƙananan sakamako masu kyau, tsakanin 14% da 50%. Duran et al. sun yi amfani da marasa lafiya 7 a cikin bincikensa (duk maza, masu shekaru 8-27) tare da LKS. A kan bin dogon lokaci, yawancin marasa lafiyarsa ba su nuna cikakkiyar farfajiya ba kuma matsalolin harshe sun ci gaba. Daga cikin marasa lafiya bakwai, daya ya ba da rahoton ingancin rayuwa na yau da kullun yayin da sauran shida suka ba da rahoton cewa aphasia ya zama gwagwarmaya mai mahimmanci. Nazarin Duran et al. yana daya daga cikin 'yan kalilan da ke nuna rahotanni na dogon lokaci na LKS kuma yana amfani da gwajin EEG, MRIs, Scale na Halin Halin Halitta na Vineland, Scale-revised na Connor, da kuma Binciken Kiwon Lafiya na Ƙananan Halitta don nazarin marasa lafiya.[10]

A duniya, an bayyana fiye da lokuta 200 na kamuwa da cutar farfajiya a cikin wallafe-wallafen. Tsakanin shekara ta 1957 zuwa 1980, an bayar da rahoton kamuwa da cututtukan farfajiya guda 81, tare da kamuwa da cutar 100 a kowace shekara 10.[1]

  1. 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 "Acquired Epileptic Aphasia" (medicinenet). Medscape Part of WebMD Health Professional Network. 2019-03-05. Cite error: Invalid <ref> tag; name "emedicine" defined multiple times with different content
  2. 2.0 2.1 "Landau-Kleffner Syndrome (LKS or Infantile Acquired Aphasia)" (medicinenet). Medscape part of WebMD Health Professional Network. Cite error: Invalid <ref> tag; name "medicinenet" defined multiple times with different content
  3. Tütüncüoğlu S, Serdaroğlu G, Kadioğlu B (October 2002). "Landau-Kleffner syndrome beginning with stuttering: case report". Journal of Child Neurology. 17 (10): 785–8. doi:10.1177/08830738020170101808. PMID 12546439. S2CID 3173640.
  4. Guevara-Campos J, González-de Guevara L (2007). "Landau–Kleffner syndrome: an analysis of 10 cases in Venezuela". Rev Neurol (in Sifaniyanci). 44 (11): 652–6. PMID 17557221.
  5. Sinclair DB, Snyder TJ (May 2005). "Corticosteroids for the treatment of Landau–Kleffner syndrome and continuous spike-wave discharge during sleep". Pediatr. Neurol. 32 (5): 300–6. doi:10.1016/j.pediatrneurol.2004.12.006. PMID 15866429.
  6. Santos LH, Antoniuk SA, Rodrigues M, Bruno S, Bruck I (June 2002). "Landau–Kleffner syndrome: study of four cases". Arq Neuropsiquiatr. 60 (2–A): 239–41. doi:10.1590/s0004-282x2002000200010. PMID 12068352.
  7. Grote CL, Van Slyke P, Hoeppner JA (March 1999). "Language outcome following multiple subpial transection for Landau–Kleffner syndrome". Brain. 122 (3): 561–6. doi:10.1093/brain/122.3.561. PMID 10094262.
  8. Morrell F, Whisler WW, Smith MC, et al. (December 1995). "Landau-Kleffner syndrome. Treatment with subpial intracortical transection". Brain. 118 ( Pt 6): 1529–46. doi:10.1093/brain/118.6.1529. PMID 8595482.
  9. Sawhney IM, Robertson IJ, Polkey CE, Binnie CD, Elwes RD (March 1995). "Multiple subpial transection: a review of 21 cases". J. Neurol. Neurosurg. Psychiatry. 58 (3): 344–9. doi:10.1136/jnnp.58.3.344. PMC 1073374. PMID 7897419.
  10. Duran MH, Guimarães CA, Medeiros LL, Guerreiro MM (January 2009). "Landau-Kleffner syndrome: long-term follow-up". Brain Dev. 31 (1): 58–63. doi:10.1016/j.braindev.2008.09.007. PMID 18930363. S2CID 25248883.

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