The efficacy of ketogenic diet in childhood intractable epilepsy with malformation of cortical development

대뇌 피질 발달 기형을 동반한 난치성 소아 간질에서 케톤생성 식이요법의 효과

  • Lee, Young-Mock (Department of Pediatrics, Institute for Handicapped Children, Yonsei University College of Medicine) ;
  • Kang, Du Cheol (Department of Pediatrics, Changwon Fatima Hospital) ;
  • Chung, Da Eun (Department of Pediatrics, Epilepsy Center, Sang-gye P aik Hospital, Inje University College of Medicine) ;
  • Kang, Hoon Chul (Department of Pediatrics, Epilepsy Center, Sang-gye P aik Hospital, Inje University College of Medicine) ;
  • Kim, Heung Dong (Department of Pediatrics, Institute for Handicapped Children, Yonsei University College of Medicine)
  • 이영목 (연세대학교 의과대학 소아과학교실, 장애아동연구소) ;
  • 강두철 (창원파티마병원 소아과) ;
  • 정다은 (인제대학교 의과대학 상계백병원 소아과, 간질센터) ;
  • 강훈철 (인제대학교 의과대학 상계백병원 소아과, 간질센터) ;
  • 김흥동 (연세대학교 의과대학 소아과학교실, 장애아동연구소)
  • Received : 2005.09.08
  • Accepted : 2005.10.25
  • Published : 2006.02.15

Abstract

Purpose : Malformation of cortical development(MCD) constitutes an important etiology of intractable epilepsy and is considered an indication for surgical treatments, though their efficacy is limited and variable depending on MCD's location or distribution. Ketogenic diets are widely known to be effective, but as little study has been made concerning their efficacy on epilepsy with MCD, we evaluated the efficacy of ketogenic diets on MCD patients compared with that of epileptic surgery, which is more invasive. Methods : We performed retrospective studies and analyse on 30 patients with MCD diagnosed by brain MRI and treated with ketogenic diets for intractable epilepsy since 1998, checking decreases in their seizure episodes after starting the diets. Results : Cortical dysplasia was observed in 24(80.0 percent) patients as the most common type of MCD. Also, MCD was observed in unilateral hemisphere most commonly, in 23(76.7 percent) patients; it was observed in both hemispheres in 7(23.3 percent) patients. Nine(30.0 percent) out of 30 patients became seizure-free after starting ketogenic diets, and 14(46.7 percent) patients experienced 50 percent seizure reductions as well. Age of starting the diet or the duration of epilepsy period before starting showed no statistical relationship with the efficacy of the diet. Though the younger the patient and the longer the treatment the more effective the diet seemed to be, there was no statistical correlation between them. The location of MCD showed no significance neither. Conclusion : Considering various limits and invasiveness of surgical treatment, a ketogenic diet could be a good tool in treating children with intractable epilepsy with MCD.

목 적 : MCD는 항경련제에 반응하지 않는 난치성 간질의 중요한 원인으로 수술적 치료의 대상으로 고려되어지지만, 병변의 범위나 분포에 따라 제한적이고 치료효과에 있어서도 차이가 보고되고 있다. 케톤생성 식이요법은 최근까지 대부분의 연구들에서 뛰어난 간질 억제효과를 보고하고 있으나, MCD 병변을 보이는 난치성 간질을 대상으로 시행한 케톤생성 식이요법의 치료 효과에 대한 보고는 국내외적으로 지금까지 없는 상태로, 본 연구에서는 이 환자군들에 대해 간질 수술보다 덜 침습적인 치료법인 케톤생성 식이요법의 간질 억제효과를 알아보고자 하였다. 방 법 : 1998년 이후 난치성 간질로 케톤생성 식이요법을 시행하였던 소아 환아들 중 뇌 MRI상 MCD 소견을 보이는 30명을 대상으로 후향적 의무기록 고찰과 분석을 시행하였으며, 케톤 생성 식이요법 시행 후 경련의 감소효과를 분석하였다. 결 과 : 전체 대상 30명 환아들의 남녀비는 1 : 1.1이었고, 경련을 처음 시작한 연령은 평균 $2.0{\pm}2.9$세, 케톤생성 식이요법을 시작한 평균 연령은 $5.4{\pm}4.6$세, 케톤생성 식이요법을 시작할 때까지의 경련 지속기간은 평균 $3.5{\pm}3.3$년, 환아들의 추적 관찰기간은 평균 $29.0{\pm}21.0$개월이었다. MCD의 종류는 대뇌 피질 이형성증(cortical dysplasia)이 24명(80.0%)으로 가장 많았고, MCD의 분포는 일측 대뇌 반구에만 있는 경우가 23명(76.7%), 양측 대뇌 반구 모두에 병변이 있는 경우가 7명(23.3%)이었다. 케톤생성 식이요법에 의한 경련의 감소 정도는 전체 30명의 환아 중 9명(30.0%)에서 경련이 완전히 소실되었으며, 50% 이상 경련이 감소된 경우는 14명(46.7%)이었는데, 경련을 처음 시작한 연령이나 케톤생성 식이요법을 시작할 때까지의 경련 지속기간은 경련의 감소효과와 통계학적 유의성이 없었고, 케톤생성 식이요법을 시작한 나이가 어릴수록, 케톤생성 식이요법의 기간이 길수록 경련의 감소효과가 높은 경향을 나타내었으나 통계적 유의성은 없었으며, MCD의 분포에 따른 특별한 연관관계는 없었다. 결 론 : 케톤생성 식이요법이 MCD 소견을 동반하고 있어 수술적 치료가 고려되어지는 난치성 소아 간질 환아에서도 간질 수술의 여러 가지 제한적인 측면과 침습성을 고려해 볼 때 효과적인 치료 결과를 기대할 수 있을 것으로 판단되며, 아직까지 국내외적으로 MCD를 동반한 난치성 소아 간질 환아에 대한 케톤생성 식이요법의 효과에 대한 자료가 미미한 상태이므로 전향적인 대규모의 비교 연구 및 분석이 필요할 것으로 사료된다.

Keywords

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