Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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소아에서 가역성 후두부 백질 뇌증 증후군의 임상적, 방사선학적 특성에 대한 고찰

Clinical and Radiological Analysis of Reversible Posterior Leukoencephalopathy Syndrome in Children

  • 임해리 (경북대학교 의과대학 소아과학교실) ;
  • 서혜은 (경북대학교 의과대학 소아과학교실) ;
  • 권순학 (경북대학교 의과대학 소아과학교실)
  • Lim, Hae-Ri (Department of Pediatrics, college of Medicine, Kyungpook National University) ;
  • Seo, Hye-Eun (Department of Pediatrics, college of Medicine, Kyungpook National University) ;
  • Kwon, Sun-Hak (Department of Pediatrics, college of Medicine, Kyungpook National University)
  • 투고 : 2007.06.22
  • 심사 : 2007.07.12
  • 발행 : 2007.09.15
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초록

목 적 : 가역성 후두부 백질 뇌증 증후군은 두정-후두엽의 백질 혹은 회질을 침범하는 특징을 가지는 복합 증후군이다. 이번 연구에서는 이 증후군의 임상적, 방사선학적 특징을 분석하였다. 방 법 : 총 15명의 환아를 대상으로 하였으며. 각 환아에서 급성기 동안의 평균 동맥압을 측정하고 신경학적 사건이 발생하기 수일에서 수주 전 안정 상태의 평균 동맥압을 조사하여 비교하였다. 그리고 이들의 뇌자기 공명 영상을 분석 비교하였으며 모든 연구는 후향적으로 이루어졌다. 결 과 : 15명의 환아(남아 : 9명, 여아 : 6명)의 연령은 2-20세(평균 연령:10세)였다. 뇌 자기공명 영상은 두정-후두엽의 백질 및 피질에 T2-weighted image에서 신호 강도의 증가를 보였다. 대개의 경우에서 스테로이드나 싸이클로스포린 복용 혹은 급격하게 혈압이 증가하는 사건과 관련성을 보였다. 결 론 : 가역성 후두부 백질 뇌증 증후군은 복합적인 신경학적 질환으로 이 복합 증후군에 대한 더 많은 연구를 함으로써 불필요한 검사를 줄이고 즉각적이고 합당한 치료를 환아에게 행할 수 있을 것으로 생각한다.

Purpose : Reversible posterior leukoencephalopathy syndrome is a complex disorder with characteristic clinical and radiologic findings that mainly involve the white/gray matter of the parieto-occipital lobes. The purpose of this study was to determine its clinical and radiological characteristics. Methods : A total of 15 pateints were involved in the study. Their medical records and radiological features of brain MRI were retrospectively reviewed and analyzed. Results : Fifteen pateints were involved. (9 males and 5 females). The patients' ages ranged from 2-20 years (mean age : 10 years). The brain MRI revealed fairly symmetric areas of increased T2 signal involving both white and gray matter of parieto-occipital regions. The condition seemed to be associated with cyclosporin A and steroid therapy or a variety of other conditions in which blood pressure rises acutely. Conclusion : Reversible posterior leukoencephalopathy syndrome is a complicated neurological condition, but a better understanding of this complex syndrome may obviate unnecessary investigations and lead to prompt and appropriate management of the associated problems.

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