Kortikosteroid Tedavisi Sonrası Otizm Spektrum Bozukluğu Bulguları Düzelen Bir Landau-Kleffner Sendromu Olgusu

Öz

Çok nadir rastlanan bir çocukluk-çağı hastalığı olan Landau-Kleffner Sendromu (LKS), elektroensefalogramdaki (EEG) epileptiform etkinlik ile ilişkili edinsel afazidir. Landau-Kleffner Sendromu’nun klinik görünümü bazı çocuklarda Otizm Spektrum Bozukluğu’na (OSB) benzeyebilir. Bu yazıda Çocuk ve Ergen Ruh Sağlığı ve Hastalıkları (ÇRS) polikliniğine OSB belirtileri ile başvuran, ancak nörolojik tetkikler sonucunda LKS tanısı konulan, LKS medikal tedavisi sonrası nöropsikiyatrik semptomlarında belirgin düzelme gösteren bir vaka sunulmuştur. Dört yaş yedi aylık erkek çocuk, konuşmada gerileme, sosyal iletişimde azalma ve tekrarlayıcı davranış kalıpları şeklindeki yakınmaları ile ÇRS polikliniğine getirilmiştir. İlk olarak OSB tanısı konulmuş ve Çocuk Nöroloji’ye yönlendirilmiş olan vakanın yapılan nörolojik değerlendirmeleri sonrasında, hastaya LKS tanısı konulmuş ve antiepileptik tedavi başlanmıştır. Üç ay kadar kullanılan konvansiyonel antiepileptik tedaviden fayda görmeyen hastaya kortikosteroid tedavi başlanmış ve 1 ay içerisinde konuşma regresyonunda ve LKS’ye bağlı olan OSB belirtilerinde klinik olarak azalma görülmüştür, 3 ay içinde ise bu belirtilerde belirgin düzelme olduğu gözlenmiştir. Bu makalede, LKS’de konvansiyonel antiepileptik tedavi ile bilişsel ve dil fonksiyonlarında ve EEG patolojisinde düzelme olmayan vakalarda kortikosteroid tedavisiyle hem nöropsikiyatrik semptomlarında hem de EEG bulgularında hızlı düzelme sağlanabileceği bildirilmiştir. Alanyazında, LKS’de bilinen tedavilerin konuşma regresyonunu düzeltmede etkili olduğuna yönelik daha fazla bilgi olmakla birlikte, LKS’ye bağlı ortaya çıkan OSB belirtilerini azaltmasına yönelik çok az vaka bildirimi vardır.

Anahtar Kelimeler

• Landau-Kleffner Sendromu
• otizm spektrum bozukluğu
• antiepileptik tedavi
• kortikosteroid tedavisi

A Case of Landau-Kleffner Syndrome with Autism Spectrum Disorder Symptoms Improved After Corticosteroid Treatment

Öz

Landau-Kleffner Syndrome (LKS), a very rare childhood disease, is acquired aphasia associated with epileptiform activity on the electroencephalogram (EEG). The clinical presentation of Landau-Kleffner Syndrome may resemble Autism Spectrum Disorder (ASD) in some children. In this article, a case who applied to the Child and Adolescent Psychiatry (CAP) outpatient clinic with ASD symptoms, but was diagnosed with LKS as a result of neurological examinations, showed significant improvement in neuropsychiatric symptoms after LKS medical treatment. A four-year-seven-month-old boy was brought to the CAP outpatient clinic with symptoms of regression in speech, impairment in social communication, and rigid and repetitive patterns of behaviour. The patient was initially diagnosed with ASD and was referred to Pediatric Neurology. After the neurological evaluations, the patient was diagnosed with LKS and antiepileptic treatment was started. Corticosteroid treatment was initiated to the patient, who did not benefit from conventional antiepileptic therapy, which was used for about three months, and a clinical decrease was observed in speech regression and ASD symptoms associated with LKS within 1 month, and a significant improvement was observed in these symptoms within 3 months. In this article, it has been reported that in cases, where there is no improvement in cognitive and language functions and also EEG pathology with conventional antiepileptic treatment in LKS, rapid improvement can be achieved in both neuropsychiatric symptoms and EEG findings with corticosteroid treatment. Although there is more information in the literature that known treatments are effective in improving speech regression in LKS, there are few case reports on reducing the symptoms of ASD associated with LKS.

Anahtar Kelimeler

• Landau-Kleffner syndrome
• autism spectrum disorders
• antiepileptic treatment
• corticosteroid treatment

Kaynakça

1. 1. Edition F. Diagnostic and statistical manual of mental disorders. Am Psychiatric Assoc. 2013;21.
2. 2. Landau WM, Kleffner FR. Syndrome of acquired aphasia with convulsive disorder in children. Neurology. 1957.
3. 3. Shah K, Rajadhyaksha S, Shah V, Shah N, Desai V. Experience with the International League Against Epilepsy classifications of epileptic seizures (1981) and epilepsies and epileptic syndrome (1989) in epileptic children in a developing country.Epilepsia. 1992;33(6):1072-1077.
4. 4. Beaumanoir A. The Landau-Kleffner syndrome. Epileptic syndromes in infancy, childhood, and adolescence. 1992:231-244.
5. 5. Duran MH, Guimarães CA, Medeiros LL, Guerreiro MM. Landau–Kleffner syndrome: long-term follow-up. Brain andDevelopment. 2009;31(1):58-63.
6. 6. Ozturk O, Ulug B. ICD-10 (International Classifıcation of Diseases-10): Classification of Mental and Behavioral Disorders. Ankara, Turkey, World Health Organization, Turkey Nervous and Mental Health Association. 1993.
7. 7. 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.
8. 8. Lainhart JE, Ozonoff S, Coon H, et al. Autism, regression, and the broader autism phenotype. American journal of medicalgenetics. 2002;113(3):231-237.

9. 9. Rapin I, Dunn M. Language disorders in children with autism. Paper presented at: Seminars in Pediatric Neurology1997.
10. 10. Bishop DV. Age of onset and outcome in ‘acquired aphasia with convulsive disorder’(Landau‐Kleffner syndrome).Developmental Medicine & Child Neurology. 1985;27(6):705-712.
11. 11. Ballaban‐Gil K, Tuchman R. Epilepsy and epileptiform EEG: association with autism and language disorders. Mental retardation and developmental disabilities research reviews.2000;6(4):300-308.
12. 12. Hughes JR, Melyn M. EEG and seizures in autistic children and adolescents: further findings with therapeutic implications. Clinical EEG and Neuroscience. 2005;36(1):15-20.
13. 13. Guilhoto L, Morrell F. Electrophysiological differencesbetween Landau-Kleffner Syndrome and other conditions showing the CSWS electrical pattern. Epilepsia. 1994;35(Suppl 8):126.
14. 14. Gordon N. The Landau-Kleffner syndrome: increasedunderstanding. Brain and Development. 1997;19(5):311-316.
15. 15. Mikati MA, Shamseddine AN. Management of Landau-Kleffner syndrome. Pediatric Drugs. 2005;7(6):377-389.
16. 16. Nass R, Gross A, Wisoff J, Devinsky O. Outcome of multiple subpial transections for autistic epileptiform regression. Pediatric neurology. 1999;21(1):464-470.
17. 17. Lerman P, Lerman‐Sagie T, Kivity S. Effect of early corticosteroid therapy for Landau‐Kleffner syndrome.Developmental Medicine & Child Neurology. 1991;33(3):257-260.
18. 18. Santos LH, Antoniuk SA, Rodrigues M, Bruno S, Bruck I.Landau-Kleffner syndrome: study of four cases. Arquivos de neuro-psiquiatria. 2002;60(2A):239-241.
19. 19. Marescaux C, Hirsch E, Finck S, et al. Landau‐Kleffner syndrome: a pharmacologic study of five cases. Epilepsia. 1990;31(6):768-777.
20. 20. Ugur C, Duman NS, Bektas O, Kagan GC. Antiepileptic Treatment in a Child With Landau Kleffner Syndrome: A Case Report. turkish journal of psychiatry. 2014;25(4).
21. 21. Alyanak B, Motavallı Mukaddes N. In this 5 case report, the EEG abnormalities in pervasive developmental disorder andoverlapping with Landau Kleffner syndrome is discussed. Dusunen Adam The Journal of Psyhiatry and NeurologicalSciences. 2002;15 (2):113-116.
22. 22. Mehra C, Sil A, Hedderly T, et al. Childhood disintegrative disorder and autism spectrum disorder: A systematic review.Developmental Medicine & Child Neurology. 2019;61(5):523-534.