Journal of the Korean Academy of Child and Adolescent Psychiatry

Korean Academy of Child and Adolescent Psychiatry (대한소아청소년정신의학회)
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A CASE OF PRADER-WILLI SYNDROME TREATED WITH FLUOXETINE

Prader-Willi 증후군의 Fluoxetine 치험 1례

  • Shin, Dong-Won (Department of Psychiatry, College of Medicine, Yonsei University) ;
  • Song, Dong-Ho (Department of Psychiatry, College of Medicine, Yonsei University)
  • 신동원 (연세대학교 의과대학 정신과학교실) ;
  • 송동호 (연세대학교 의과대학 정신과학교실)
  • Published : 1997.06.30
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Abstract

Prader Willi Syndrome(PWS) was first recognized and reported by Prader-Willi. The etiology of the syndrome is not fully understood, but 50-70% of the patients show small deletion in chromosome 15. Manifested symtoms vary according to developmental age. In early life, hypotonia, areflexia, feeding difficulties, hypothermia, microgenitalia, hypoplastic scrotum, cryptochordism were observed. But in several years, hypotonia disappears, and polyphagia, decreased satiety, psychomotor retardation, obesity, hypogonadism and short stature become main problems. Behavioural problems including temper and aggressive outbursts, stealing food, hoarding food, and self excoriating skin picking, trichotillomania are more prominent during adolescence and young adulthood. Also, irritable, depressed mood are described. Lots of psychological and behavioural problems explain the reason why psychiatrists have managed and reported this syndrome. However, there has been no official report of PWS in our country. So authors report the clinical characteristics and issues in management of a patient with PWS.

Prader-Willi 증후군(Prader-Willi Syndrome, PWS)은 $50{\sim}-70%$ 정도의 환자에서 염색체 15번에 이상이 있음이 보고된 유전적인 질환으로서, 그 특징적인 임상양상은 심한 근긴장의 저하(hypotonia), 반사소실(areflexia), 섭식의 곤란(feeding difficulty), 저체온증(hypothermia), 성기왜소증(microgenitalia), 음낭저형성증(hypoplastic scrotum), 대식증(polyphagia), 포만감의 감소와 비만, 정신운동발달의 지연, 저성선기능증(hypogonadism) 및 안면과 척추의 기형 등이다. 또한 행동상의 여러 문제들이 동반되는데 분노발작, 피부를 심하게 뜯거나 발모광, 과도한 식욕과 관련된 음식 도벽증이 나타나므로 정신과적 치료가 필요하다. 본 증례는 14세된 PWS환자로서 비만과 대식증, 도벽증, 학업부진 및 생활부적응 문제 등으로 2주간의 입원과 약 1년간의 추적기간 동안 약물요법(fluoxetine 투여)과 행동요법, 그리고 가족요법을 받아 왔다. 장기간의 Fluoxetine투여를 통하여 비만과 식욕의 조절에는 효과가 만족스럽지 않았으나 우울감과 감정문제, 자살사고 및 행동문제들은 호전되었다.

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