Behavioral Variant Frontotemporal Dementia Phenocopy Syndrome

Cheon, Jin Sook

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.