Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Long-term outcomes of infantile spasms

영아 연축 환아의 장기적 예후에 관한 고찰

  • Oh, Seak Hee (Department of Pediatrics, Asan Medical Center Children's Hospital University of Ulsan College of Medicine) ;
  • Lee, Eun-Hye (Department of Pediatrics, Asan Medical Center Children's Hospital University of Ulsan College of Medicine) ;
  • Joung, Min-Hee (Department of Pediatrics, Asan Medical Center Children's Hospital University of Ulsan College of Medicine) ;
  • Yum, Mi-Sun (Department of Pediatrics, Asan Medical Center Children's Hospital University of Ulsan College of Medicine) ;
  • Ko, Tae-Sung (Department of Pediatrics, Asan Medical Center Children's Hospital University of Ulsan College of Medicine)
  • 오석희 (울산대학교 의과대학 서울아산병원 소아과학교실) ;
  • 이은혜 (울산대학교 의과대학 서울아산병원 소아과학교실) ;
  • 정민희 (울산대학교 의과대학 서울아산병원 소아과학교실) ;
  • 염미선 (울산대학교 의과대학 서울아산병원 소아과학교실) ;
  • 고태성 (울산대학교 의과대학 서울아산병원 소아과학교실)
  • Received : 2009.07.15
  • Accepted : 2009.10.26
  • Published : 2010.01.15
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Abstract

Purpose : The aims of this study were to investigate the long-term outcomes in children with infantile spasms (IS) and to identify the prognostic factors influencing their neurodevelopment. Methods : We retrospectively evaluated seventy two children over five years old who were treated for IS at Asan Medical Center, Seoul, Korea, between 1994 and 2007. Forty-three children were contacted by telephone or medical follow-up to assess their current neurodevelopmental status. Multiple logistic regression was used to calculate odds ratios (ORs) and 95% confidence interval (95% CIs) of risk factors for unfavorable outcomes.Results : The mean follow-up duration for these 43 children was $7.2{\pm}1.5$ years (range, 4.5 to 13.0 years). Of these, 13 (30.2%) had cryptogenic and 30 (69.8%) had symptomatic IS. Eleven (25.6%) children were initially treated with adrenocorticotrophic hormone (ACTH) therapy, with a mean treatment lag of $1.3{\pm}1.9$ months (range; 0.1 to 7.0 months). Eighteen (41.8%) children clinically responded to initial treatment, as shown by EEG response. Overall, 22 (51.2%) children had at least moderate neurodevelopmental disorders and 2 (4.8%) died. In univariate analysis, etiology (symptomatic) and poor electroclinical response to initial treatment were related to long-term unfavorable outcomes. In multivariate analysis, response to primary treatment was the sole significant independent risk factor with a high OR. Conclusion : Overall prognosis of children with IS was poor. Electroclinical non-responsiveness to initial treatment was related to unfavorable long-term outcomes, indicating that initial control of seizures may be important in reducing the likelihood of poor neurodevelopment.

목 적 : 이 연구의 목적은 영아 연축 환아들의 장기적 예후를 분석하며 그들의 신경학적 발달지연에 영향을 주는 예후인자들을 찾고자 한다. 방 법 : 저자들은 1994년에서 2007년까지 서울아산병원에서 영아 연축으로 진단받은 72명의 소아들에 대하여 후향적 분석을 시행하였다. 이들 중 43명에 대한 전화 상 질문서 및 의무기록을 통하여 현재의 신경학적 발달에 대한 평가를 시행하였다. 불량한 장기적 예후와 관련된 인자들의 확인을 위해 로지스틱 회귀분석을 사용하였다. 결 과 : 13명(30.2%)이 특발성이며 30명(69.8%)이 증후성이었다. 11명(25.6%)이 ACTH 치료에 반응하였으며, 치료지연은 $1.3{\pm}1.9$개월이었다. 18명(41.8%)이 초기치료에 호전된 반응을 보였으며, 평균 추적기간은 $7.2{\pm}1.5$년이었다. 장기 예후 분석에서는 22명(51.2%)이 중등도 이상의 신경학적 발달지연을 보였으며, 그 중 2명(4.8%)이 사망하였다. 단변량 분석에서는 증후성 영아 연축과 초기 치료에 불량한 반응이 중등도 이상의 신경학적 발달지연과 관련된 유의한 위험인자였으나, 다변량 분석에서는 초기 치료에 불량한 반응만이 위험인자로 확인되었다. 결 론 : 영아 연축 환아의 전반적인 예후는 불량하였고 초기치료에 불량한 반응을 보인 환자군에서 장기적 예후가 더욱 불량하였다. 이러한 결과는 경련의 초기 조절이 영아 연축 환아에서 신경학적 발달지연과 관련된 장기적 예후에 영향을 줄 수 있음을 시사한다.

Keywords

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