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Çocuklugun dezintegratif bozuklugu çok nadir görülen bir yayg?n gelisimsel bozukluktur. Ilk kez T . Heller taraf?ndan 1908 y?l?nda infantil demans ad?yla tan?mlanm?st?r. Yasam?n ilk 3-4 y?l?nda normal kabul edilen gelisimin ard?ndan sosyal ve iletisim becerilerinde ciddi gerileme olmas? bozukluk için tipiktir. Tahmin edilen yayg?nl?g? 100.000 çocukta 1 civar?ndad?r. Cinsiyet oran? bilinmemesine ragmen, erkek olgular?n say?s? k?zlardan daha yüksek gibi görünmektedir. Prognoz genellikle kötüdür ve bireylerin çogunda özellikle dil ve sosyal beceriler konu - sunda ciddi gerilik vard?r. Bozuklugun dogas? ve diger yayg?n gelisimsel bozukluklarla iliskisi henü z bilinmemektedir. Nörolojik hastal?klar ve diger t?bbi sorunlar bu bozuklukla iliskili olabilir. Burada yaklas?k üç y?ll?k normal gelisimin ard?ndan çocukluk çag? dezintegratif bozukluk gelisen bir olgu sunulmustur. Çocugun davran?s sorunla - r?n?n anne kayb?n?n ard?ndan baslad?g? belirtilmistir. EEG ve beyin MR incelemeleri sonucunda nörolojik bir anormallik saptanmad?. Tedavi boyunca belirtiler antipsikotik ilaç kullan?larak kontrol alt?na al?nd?. (Anadolu Psikiyatri Derg. 2013;14:174-7)
Anahtar sözcükler: Çocukluk çag? dezintegratif bozuklugu, klinik özellikler, sonlanma
ABSTRACT
Childhood disintegrative disorder is a rare pervasive disorder. It was first reported by Theodor Heller under the name of dementia infantilis in 1908. Severe regression in social and communication skills occurs after apparently normal development in the first 3-4 years of life. Estimated prevalence of Childhood disintegrative disorder is around 1 per 100.000 children. Although gender distrubition of the disorder is not exactly known, male seems to outnumber female cases. The prognosis is usually very poor and most individuals are left with severe developmental delay especially in language and social skills. The nature of the illness and its relation with other pervasive developmental disorders has not been solved yet. Neurological disorders and other medical conditions are sometimes associated with this disorder. The present report describes a case that developed childhood disintegrative disorder after approximately three years of normal development. The child"s behavioral problems reportedly began at that age following the death of his mother. No neurological abnormalities were found as a result of EEG and serebral MRI. During the treatment symptoms have been controlled by antipsychotic drugs. (Anatolian Journal of Psychiatry. 2013;14:174-7)
Key words: childhood disintegative disorder, clinical features, prognosis
GIRIS
Çocuklugun dezintegratif bozuklugu ilk kez 1908"de Heller Sendromu olarak tan?mlanm?Gt?r. Ard?ndan literatürde infantil demans, ilerleyici dezintegratif psikoz, dezintegratif psikoz, yayg?n dezintegratif bozukluk, geç baGlang?çl? otizm...