Tamer ERDEM, Tayfun KİRAZLI, Sarenur TÜTÜNCÜOĞLU

Landau-Kleffner sendromu edinsel epileptik afazi

Landau-Kleffner sendromu konuflma yetisinin bozul- ması, epilepsi nöbetleri ve anormal elektroensefa- lografi bulgularıyla kendini gösteren bir epilepsi türü- dür. Bu yazıda, üç yıldır konuflma zorluğu çeken ve son iki yıldır da jeneralize tonik-klonik nöbetleri olan altı yaflındaki bir erkek olgu sunuldu. Tonal odyomet- ri ve beyin sapı odyometrisinde anormal bulguya rastlanmadı. Hastada gerçek bir konuflamama afa- zi değil, sözlü iflitsel uyaranların ayırt edilemeyifli ve anlaflılamaması sözel-iflitsel agnozi vardı. Kranyal bilgisayarlı tomografi ve manyetik rezonans görüntü- leme bulguları normaldi. Single-photon emisyonlu bilgisayarlı tomografide sağ hemisfer temporal lob posteroinferior kısmında hipoperfüzyon bulgularına rastlandı. Kortikosteroid ve intravenöz immünglobu- lin tedavisine rağmen dört yıllık izlem sırasında has- talıkta dalgalı bir seyir gözlendi

Anahtar Kelimeler:

Antikonvulsan/terapötik kullanım, afazi/tanı/komplikasyon, çocuk, elektroensefalografi, epilepsi/komplikasyon, Landau-Kleffner sendromu/tedavi, konuflmabozuklukları/etyoloji, nöbet/komplikasyon, sendrom

Landau-Kleffner syndrome acquired epileptic aphasia

Landau-Kleffner syndrome is characterized by a complex group of symptoms including deterioration in language skills, seizures, and abnormal elec- troencephalography findings. A six-year-old male patient had aphasia for three years and generalized tonic-clonic epileptic seizures for two years. Pure- tone audiometry and auditory brain-stem response audiometry findings were normal. He had verbal auditory agnosia rather than true aphasia. Cranial computed tomography and magnetic resonance imaging studies did not show any abnormal findings.Single-photon emission computed tomography showed hypoperfusion in the right hemispheric tem poral lobe. Despite treatment with corticosteroids and intravenous immunoglobulins, multiple exacerbations were interspersed in a four-year follow-up period.

Keywords:

Anticonvulsants/therapeutic use, aphasia/diagnosis/complications, child, electroencephalography, epilepsy/ complications, Landau-Kleffner syndrome/therapy, language disorders/etiology, seizures/complications, syndrome,

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Wannag E. The Landau-Kleffner syndrome. In: Sillanpaa M, Johannessen SI, Blennow G, Dam M, edi- tors. Paediatric epilepsy. 1st ed. Petersfield: Wrightson Biomedical Publishing; 1990. p. 123-8.
Cole AJ, Andermann F, Taylor L, Olivier A, Rasmussen T, Robitaille Y, et al. The Landau-Kleffner syndrome of acquired epileptic aphasia: unusual clinical outcome, s u rgical experience, and absence of encephalitis. Neurology 1988;38:31-8.
Deonna T, Peter C, Ziegler AL. Adult follow-up of the a c q u i red aphasia-epilepsy syndrome in childhood. Report of 7 cases. Neuropediatrics 1989;20:132-8.
Zouari N, Choyakh F. Early, middle-latency and late auditory evoked potentials in a case of acquire d epileptic aphasia (Landau-Kleffner syndrome). Rev Laryngol Otol Rhinol 1997;118:267-70. [Abstract]
Gascon G, Victor D, Lombroso CT. Language disor- ders, convulsive disorder, and electroencephalograph- ic abnormalities. Acquired syndrome in children. Arch Neurol 1973;28:156-62.
Gordon N. The Landau-Kleffner syndrome: increased understanding. Brain Dev 1997;19:311-6.
Guerreiro MM, Camargo EE, Kato M, Menezes Netto JR, Silva EA, Scotoni AE, et al. Brain single photon emission computed tomography imaging in Landau- Kleffner syndrome. Epilepsia 1996;37:60-7.
da Silva EA, Chugani DC, Muzik O, Chugani HT. Landau-Kleffner syndrome: metabolic abnormalities in temporal lobe are a common feature. Child Neurol 1997;12:489-95.
Mantovani JF, Landau WM. Acquired aphasia with convulsive disorder: course and prognosis. Neurology 1980;30:524-9.
Ravnik I. A case of Landau-Kleffner syndrome: effect of intravenous diazepam. In: Roger J, Dravet C, Bureau M, Dreifuss FE, Wolf P, editors. Epileptic syn- dromes in infancy, childhood, and adolescence. 1st ed. London: John Libbey Eurotext; 1985. p. 192-3.
Fayad MN, Choueiri R, Mikati M. Landau-Kleffner syn- d rome: consistent response to repeated intravenous gamma-globulin doses: a case report. Epilepsia 1997;38: 4 8 9 - 9 4 .
Lagae LG, Silberstein J, Gillis PL, Casaer P. Successful use of intravenous immunoglobulins in Landau- Kleffner syndrome. Pediatr Neurol 1998;18:165-8.
Neville BG, Harkness WF, Cross JH, Cass HC, Burch VC, Lees JA, et al. Surgical treatment of severe autistic regression in childhood epilepsy. Pediatr Neurol 1997; 16:137-40.
Morrell F, Whisler WW, Smith MC, Hoeppner TJ, de To l e d o - M o r rell L, Pierre-Louis SJ, et al. Landau-Kleff n e r syndrome. Treatment with subpial intracortical transec- tion. Brain 1995;118(Pt 6):1529-46.

The Turkish Journal of Ear Nose and Throat-Cover

ISSN: 2602-4837
Yayın Aralığı: Yılda 4 Sayı
Başlangıç: 1991
Yayıncı: İstanbul Üniversitesi

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