CAJ | 학술논문

目的探讨行为异常型额颞叶痴呆(bvFTD)患者的临床及影像学特点.方法对2007年1月至2013年9月登记的38例bvFTD及22例阿尔茨海默病(AD)患者的临床及影像学资料进行回顾性分析,收集了包括Addenbrooke改良认知评估量表(ACE-R)、额叶行为量表(FBI)、额叶功能量表(FAB)的评估结果(分)以及MRI检查结果,并进行比较.结果 (1)bvFTD早期最常见的症状依次为执行功能障碍、行为去抑制、淡漠、同情心丧失、记忆力下降、口欲亢进、强迫化行为,误诊率高(31/38).(2)bvFTD患者与AD患者在发病年龄上差异有统计学意义,性别、教育程度、病程差异无统计学意义.bvFTD组与AD组相比,在ACE-R [(38.0±19.8)分与(25.7±15.9)分]、FBI[(30.9±10.0)分与(17.8±7.6)分]、FAB[(8.0±3.1)分与(9.7±2.6)分]评估结果上差异有统计学意义(t=2.472、5.313、-2.127,均P＜0.05).FBI量表诊断bvFTD最佳划分界值为24.5分,敏感度为76.3％,特异度为95.5％.(3)bvFTD组患者头颅MRI表现为双侧大脑半球前部对称或不对称性额颞叶萎缩,萎缩的程度及范围变异大,8例患者伴有不同程度海马萎缩.结论 bvFTD患者早期症状复杂,易误诊,首发症状多为行为、执行功能障碍.成套行为-认知量表有助于bvFTD诊断.与AD相比,bvFTD的典型MRI改变为前脑(额颞叶)萎缩.
목적 탐토행위이상형액섭협치태(bvFTD)환자적림상급영상학특점.방법 대2007년1월지2013년9월등기적38례bvFTD급22례아이자해묵병(AD)환자적림상급영상학자료진행회고성분석,수집료포괄Addenbrooke개량인지평고량표(ACE-R)、액협행위량표(FBI)、액협공능량표(FAB)적평고결과(분)이급MRI검사결과,병진행비교.결과 (1)bvFTD조기최상견적증상의차위집행공능장애、행위거억제、담막、동정심상실、기억력하강、구욕항진、강박화행위,오진솔고(31/38).(2)bvFTD환자여AD환자재발병년령상차이유통계학의의,성별、교육정도、병정차이무통계학의의.bvFTD조여AD조상비,재ACE-R [(38.0±19.8)분여(25.7±15.9)분]、FBI[(30.9±10.0)분여(17.8±7.6)분]、FAB[(8.0±3.1)분여(9.7±2.6)분]평고결과상차이유통계학의의(t=2.472、5.313、-2.127,균P＜0.05).FBI량표진단bvFTD최가화분계치위24.5분,민감도위76.3％,특이도위95.5％.(3)bvFTD조환자두로MRI표현위쌍측대뇌반구전부대칭혹불대칭성액섭협위축,위축적정도급범위변이대,8례환자반유불동정도해마위축.결론 bvFTD환자조기증상복잡,역오진,수발증상다위행위、집행공능장애.성투행위-인지량표유조우bvFTD진단.여AD상비,bvFTD적전형MRI개변위전뇌(액섭협)위축.
Objective To explore the clinical and neuroimaging features of behavioral variant of frontotemporal dementia (bvFFD).Methods Thirty-eight patients with bvFTD compared with 22Alzheimer's disease (AD) patients were retrospectively evaluated by clinical and neuroimaging features from January 2007 to September 2013.The Addenbrooke's Cognitive Examination-revised (ACE-R),Frontal Behavioral Inventory (FBI),and Frontal Assessment Battery (FAB)were applied for cognitive and behavioral performances together with MRI.Results (1) The most common symptoms of bvFTD were followed by executive deficits,behavioral disinhibition,apathy,loss of sympathy,memory decline,hyperorality and compulsive behaviour.The misdiagnosis rate was high (31/38).(2) The age of bvFTD group was younger than AD group,but there were no significant differences in education,sex ratio and the duration of symptoms between two groups.And there were significant differences in the scores of ACE-R(38.0 ± 19.8 vs 25.7 ±15.9,t=2.472,P ＜0.05),FBI (30.9 ± 10.0 vs 17.8 ±7.6,t =5.313,P ＜0.01),FAB(8.0 ±3.1 vs 9.7 ±2.6,t =-2.127,P ＜0.05) between two groups.The optimal FBI cut-off score of 24.5 gave 76.3％sensitivity and 95.5％ specificity in distinguishing bvFTD from AD patients.(3) MRI studies of bvFTD patients revealed symmetrical or asymmetrical atrophy of the frontal and/or temporal lobes.The pattern of atrophy varies significantly.The atrophy of hippocampus was showed in 8 cases of bvFTD patients.Conclusions The early symptoms of bvFTD patients are complex,and easily misdiagnosed.Onset symptoms are mostly behavioral disinhibition,executive deficits,combination of behavioral and cognitive scales promotes early diagnosis.MRI studies show that bvFTD presents with a combination of anterior frontal and temporal cortical atrophy.