Landau-Kleffner Sendromu
Özet
Landau-Kleffner Sendromu (LKS), çocukluk çağında dil gelişiminin durması veya gerilemesiyle karakterize, genellikle uyku sırasında yoğunlaşan epileptiform aktivitenin eşlik ettiği nadir bir nörolojik bozukluktur. Hastalarda alıcı ve ifade edici dil yeteneklerinde kayıplar görülürken, çoğu vakada işitsel agnozi ve eşlik eden davranışsal sorunlar tabloya dahil olmaktadır. Tanı, klinik gözlem ve uyku EEG'si ile konulmakta, tedavi ise genellikle antiepileptik ilaçlar ve kortikosteroidler ile yürütülmektedir. Hastalığın seyri değişkenlik gösterip, tamamen iyileşmeden kalıcı dil bozukluğuna kadar geniş bir prognoz aralığına sahiptir.
Landau-Kleffner Syndrome (LKS) is a rare childhood neurological disorder characterized by the sudden or progressive loss of language skills, accompanied by epileptiform activity that intensifies during sleep. Patients typically exhibit profound receptive and expressive language deficits, often complicated by auditory agnosia and behavioral issues, even in the absence of clinical seizures. Diagnosis relies on clinical history and EEG findings, with treatment primarily involving antiepileptic medications and corticosteroids to suppress the epileptiform activity. Prognosis is highly variable, ranging from complete recovery of language abilities to permanent impairment depending on the age of onset and duration of the regression.
Referanslar
Landau WM, Kleffner FR. Syndrome of acquired aphasia with convulsive disorder in children. Neurology 1957;7:523-530.
Tuft M, Arva M, Bjornvold M, Wilson JA, Nakken KO. Landau-Kleffner syndrome. Tidsskr Nor Laegeforen. 2015;135(22):2061-2064.
Kaga M, Inagaki M, Ohta R. Epidemiological study of Landau-Kleffner syndrome. Brain Dev. 2014;36(4):284-286.
Clark M, Holmes H, Ngoh A, Siyani V, Wilson G. Overview of Landau-Kleffner syndrome: early treatment, tailored education and therapy improve outcome. Paediatrics and Child Health 2021;31(5):207-219.
Caraballo RH, Crejas N, Chamorro N, Kaltenmeier MC, Fortini S, Soprano AM. Landau-Kleffner syndrome: A study of 29 patients. Seizure 2014;23(2):98-104.
Fandino M, Connoly M, Usher L, Palm S, Kozak FK. Landau-Kleffner syndrome: A rare auditory processing disorder series of cases and review of the literature. International J Pediatr Otorhinolaryngology 2011;75(1):33-38.
Chen WX, Yang SD, Gao YY, Ning SY, Peng BW, Zhang YN. Landau Kleffner Syndrome: An Unusual Case With Progressive Ataxia Prior To Language Regression And Autistic-Like Behaviors.
Neuropsychiatry 2019;9(1):2077-2085.
Denes G. Acquired epileptiform aphasia or Landau-Kleffner Syndrome: Clinical and Linguistic Aspects. In B. Stenner (Ed.). Handbook of the Neuroscience of Language 2008 (pp 361-366), Boston MA: Academic Press
Mikati MA, Kurdi RM, Shamseddine AN. Landau Kleffner Syndrome. In Whitaker H. Concise Encyclopedia of Brain and Language 2009 (pp 259-263), Amsterdam, Elsevier Ltd.
Stefanatos GA, Kinsbourne M, Wasserstein J. Acquired epileptiform aphasia: A dimensional view of Landau-Kleffner syndrome and the relation to regressive autistic spectrum disorders. Child Neuropsychology 2002;8(3):195-228.
Yang X, Qian P, Xu X, Liu X, Wu X, Zhang Y, et al. GRIN2A mutations in epilepsy-aphasia spectrum disorders. Brain Dev. 2018;40(3):205-210.
Takeoka M, Riviello JJ, Duffy FH, Kim F, Kennedy DN, Makris N, et al. Bilateral volume reduction of the superior temporal areas in Landau-Kleffner syndrome. Neurology 2004;63(7):1289-1292.
Mikati MA, Shamseddine AN. Management of Landau-Kleffner syndrome. Paediatr Drugs. 2005;7(6):377-389.
Hughes J. A review of the relationship between Landau-Kleffner syndrome, electrical status epielpticus during sleep, and continuous spike-waves during sleep. Epilepsy Behav 2011;20(2):247-253.
Mikati MA, Saab R. Succesful use of intravenous immunoglobulin as initial monotherapy in Landau-Kleffner syndrome. Epilepsia 2000;41(7):880-886.
Smith MC, Spitz MC. Treatment strategies in Landau-Kleffner syndrome and paraictal psychiatric and cognitive disturbances. Epilepsy Behav 2002;3(5S):24-29.
Holmes GL, Riviello Jr JJ. Treatment of childhood idiopathic language deterioration with valproate. Epilepsy Behav 2001;2(39):272-276.
Verhelst H, Boon P, Buyse G, Ceulemans B, D’Hooghe M, Meirleir LD,et al. Steroids in intractable childhood epielspy: clinical experience and review of the literature. Seizure 2005;14(6):412-421.
Arts WF, Aarsen FK, Scheltens-de Boer M, Catsman-Berrevoets CE. Landau-Kleffner syndrome and CSWS syndrome: treatment with intravenous immunoglobulins. Epilepsia 2009;50 Suppl 7:55-58.
Mikati MA, Saab R, Fayad MN, Choueiri. Efficacy of intravenous immunoglobulin in Landau-Kleffner syndrome. Pediatr Neurol 2002;26(49):298-300.
Cross JH, Neville BGR. The surgical treatment of Landau-Kleffner syndrome. Epilepsia 2009;50(Suppl. 7):63-67.
Grote CL, Van Slyke P, Hoeppner JA. Language outcome following multiple subpial transection for Landau-Kleffner syndrome. Brain. 1999;122 ( Pt 3):561-566.
Downes M, Greenaway R, Clark M, Cross JH, Jolleff N, Harkness W, et al. Outcome following multiple subpial transection in Landau-Kleffner syndrome and related regression. Epilepia 2015;56(11):1760-1766.
Park YD. The effects of vagus nerve stimulation therapy on patients with intractable seizures and either Landau-Kleffner syndrome or autism. Epilepsy Behav. 2003;4(3):286-290.
Duran MHC, Guimaraes CA, Mediros LL, Guerreiro MM. Landau-Kleffner syndrome: Long-term follow-up. Brain Dev 2009;31(1):58-63.
Rossi PG, Parmeggiani A, Posar A, Scaduto MC, Chiodo S, Vatti G. Landau-Kleffner syndrome (LKS): long-term follow-up and links with electrical status epilepticus during sleep (ESES). Brain Dev. 1999;21(2):90-98.
