Şakir ALTUNBAŞAK, M. Özlem HERGÜNER, Faruk İNCECİK

Landau-Kleffner sendromu: İki olgu sunumu

Landau-Kleffner sendromu veya edinsel epileptik konuşamama (afazi) normal dil ve motor gelişimi gösteren çocuklarda ortaya çıkan kazanılmış bir konuşamama halidir. Henüz bilinen bir tedavisi olmamasına rağmen, şimdiye kadar valproik asit, yüksek doz metil-prednizolon, adrenokortikotropin hormon, damar içi immünglobülin, klobazam, sultiyam, levatirasetam ve cerrahi tedavi (mutiple subpial transection) kullanılmış ve başarılı sonuçlar bildirilmiştir. Biz de beş ve sekiz yaşında Landau-Kleffner sendromu tanısı konulan iki olgumuza valproik asit ile birlikte önce damar içi immünglobülin, yanıt alamadığımız için sonra yüksek doz metil-prednizolon uyguladık. Damar içi immünglobülin tedavisine iki olgumuzda da yanıt alamadık. Ancak yüksek doz metil prednizolon uyguladığımız olgularımızdan birisinde hem klinik, hem de elektroansefalogram bulgularında düzelme saptadık.

Landau-Kleffner syndrome: two cases report

Landau-Kleffner syndrome (LKS) or acquired epileptic aphasia is an acquired aphasia in children showing normal language and motor development. Although its treatment is not standardized till now valproic acid (VPA), high dose methyl-prednisolone, adrenocorticotropic hormone, intravenous immunoglobulin (IVIG), clobazam, sulthiam, levatiracetam and surgical treatment (multiple subpial transections) were used and successful results were reported. We just administered high doses of methyl-prednisolone to our five and eight years old two cases of Landau-Kleffner syndrome as we couldn’t recieve response to valproic acid in addition to intravenous immungloubulın treatment. However of the cases who received high dose methyl-prednisolone showed clinical and elektroencephalographical improvement.

PDF

___

1. Landau WM, Kleffner FR. Syndrome of acquired aphasia with convulsive disorder in children. Neurology 1957; 7: 523-30.
2. Bharani S, Trivedi C, Shendurnikar N, et al. Landau Kleffner syndrome. Indian J Pediatr 2001; 68: 567-9.
3. Tsuru T, Mori M, Mizuguchi M, et al. Case reports. Effects of high-dose intravenous corticosteroid therapy in Landau-Kleffner syndrome. Pediatr Neurol 2000; 22: 145-7.
4. Mikati M, Fayad M, Choueri R. IVIG in Landau-Kleffner syndrome. Pediatr Neurol 1998; 19: 399-400.
5. Marescaux C, Hirsch E, Finck S, et al. Landau-Kleffner syndrome: A pharmacologic study of five cases. Epilepsia 1990; 31: 768–77.
6. Wakai S, Ito N, Ueda D, et al. Landau-Kleffner syndrome and sulthiame. Neuropediatrics 1997;28:135-6.
7. Irwin K, Birch V, Lees J, et al. Multiple subpial transection in Landau Kleffner syndrome. Dev Med Child Neurol 2001; 43: 248-52.
8. Mikati MA, Shamseddine AN. Management of Landau-Kleffner syndrome. Paediatr Drugs 2005; 7: 377-89.
9. Kossoff EH, Boatman D, Freeman JM. Landau-Kleffner syndrome responsive to levetiracetam. Epilepsy Behav 2003; 4: 571-5.
10. Mikati MA, Saab R, Fayad MN, et al. Efficacy of intravenous immunoglobulin in Landau-Kleffner syndrome. Pediatr Neurol 2002;26:298-300.
11. Kirkham K. Diagnosis, investigations and initation of treatment in childhood epilepsies. In: Telly Meldrum BS, (eds). Recent Advances in Epilepsy. London: Publ Churchill Livingstone, 1995: 141-70.
12. Fayad MN, Choueiri RN, Mikati M. Landau-Kleffner syndrome: consistent response to repeated ıntravenous gamma–globulin doses: A case report. Epilepsia 1997; 38: 489-94.
13. Lerman P, Lerman-Sagie T, Kivity S. Effect of early corticosteroid therapy for Landau-Kleffner syndrome. Dev Med Child Neurol 1991; 33: 257–60.
14. Guevara-Campos J, González-de Guevara L. Landau-Kleffner syndrome: an analysis of 10 cases in Venezuela. Rev Neurol 2007; 44: 652-6.
15. Gallagher S, Weiss S, Oram Cardy J, et al. Efficacy of very high dose steroid treatment in a case of Landau-Kleffner syndrome. Dev Med Child Neurol 2006; 48: 766-9.