Korean Journal of Psychosomatic Medicine (정신신체의학)

Korean Psychosomatic Society (한국정신신체의학회)
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Behavioral Variant Frontotemporal Dementia Phenocopy Syndrome

행동증상 아형 전측두엽 치매 표현형모사 증후군

  • Cheon, Jin Sook (Department of Psychiatry, Kosin University College of Medicine)
  • 전진숙 (고신대학교 의과대학 정신건강의학교실)
  • Received : 2016.12.12
  • Accepted : 2017.02.21
  • Published : 2017.06.30
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Abstract

Objectives : The aim of this study was to draw attention toward so called 'behavioral variant frontotemporal dementia(bvFTD) phenocopy syndrome', which is difficult to discriminate with the primary psychiatric disorders, showing poor response to conventional therapeutic drugs, leading to higher risk to misdiagnoses and legal problems. Furthermore, the author insisted that our interest and study on them must be continued. Methods : English articles published during 2000 thru 2016 had been searched by internet with the combination of words such as 'frontotemporal', 'phenocopy' and 'behavioral', and reviewed. Besides, two clinical vignettes were described. Results : Precise diagnosis is important because patients' behavioral symptoms can influence on their families and community. However, disease-modifying treatment for bvFTD are not developed until now, and recent therapeutic drugs are only good for specific symptoms, while deterioration progresses in spite of proper psychiatric management. The possible bvFTD patients are not progressed into probable bvFTD clinically, showing no decline of cogntive and social function, no decrease of activity function, longer survival time, and normal neuroimaging for several years. Conclusions : Rather than expected, there are much more patients having clinical symptoms, course and diagnostic findings including neuroimaging, which are atypical to classical frontotemporal dementia and primary psychiatric disorders. If our knowledge and discriminating ability is improved, discovery rate of that cases will be increased. However, the identity of these atypical features are not clarified until now, it must be further actively investigated.

연구목적 소위 '행동증상 아형 전두측두엽 치매 표현형모사 증후군(behavioral variant frontotemporal dementia phenocopy syndrome)' 환자들은 일차성 정신장애와 감별이 힘들고, 기존의 치료 약물에 대한 반응도 회의적이어서, 오진의 위험과 법적 문제의 유발 가능성이 높으므로, 이러한 양상을 보이는 환자군에 대한 지속적인 관심과 연구가 필요함을 환기 시키기 위해서 본 연구를 시행하였다. 방법 2000년 부터 2016년 까지 발행된 영문 학술지에 게재된 논문을 인터넷에서 'frontotemporal', 'phenocopy', 'behavioral'과 같은 단어의 조합으로 검색하여 찾은 참고문헌을 정리, 고찰하였다. 또한 저자가 직접 경험한 두 증례를 간략히 기술하였다. 결과 환자의 행동 증상은 가족과 지역사회에 큰 영향을 끼치므로 행동증상 아형 전두측두엽 치매에서 정확한 진단이 중요하다. 그러나, 행동증상 아형 전두측두엽 치매에서 현재까지 질병 수정 치료법은 개발되지 않았으며, 현재의 약물학적 치료는 특수한 증상에 도움이 될 뿐이고, 적절한 정신의학적 치료에도 불구하고 점진적인 퇴행이 진행된다. 상당수의 '행동증상 아형 전두측두엽 치매 의심(possible bvFTD)' 환자에서 임상적으로 '행동증상 아형 전두측두엽 치매 가능(probable bvFTD)'으로 진행되지 않으며, 인지기능이나 사회적 기능이 저하되지 않고, 활동 기능의 저하를 보이지 않으며, 생존기간이 조금 더 길고, 수년에 걸쳐서 정상적인 뇌영상화 검사 소견을 보인다. 결론 환전두측두엽 치매 및 일차성 정신장애 환자에서 보이는 전형적인 임상적 양상이나 경과, 뇌영상화 검사를 포함한 진단적 평가와는 다른 비전형적인 소견을 보이는 환자군들은 생각보다 많으며, 정신의학과 의사들이 이에 대한 의학적 지식과 판별 능력이 발전되면, 그 발견의 비율은 훨씬 더 높아질 것이다. 그러나, 현재로서는 이러한 비전형적인 환자군의 정체에 대해서는 이견이 많으며, 향후 적극적인 연구가 행해져야할 분야이다.

Keywords

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