行为异常型额颞叶痴呆38例临床和影像学特点  

Clinical features and imaging analysis of 38 cases of behavioral variant frontotemporal dementia

作　　者：顾小花[1] 程欣欣[1] 张冰[2] 张鑫[2] 韦存胜[1] 武文博[2] 李冠军[3] 蔡荇[1] 徐俊[4] 

机构地区：[1]南京医科大学附属脑科医院神经内科,210029 [2]南京大学医学院附属鼓楼医院医学影像科 [3]上海市精神卫生中心老年精神科 [4]南京医科大学附属江苏省老年医院神经内科,210024

出　　处：《中华神经科杂志》2014年第5期299-304,共6页Chinese Journal of Neurology

基　　金：国家自然科学基金资助项目（81271211,30700248）;科技部新药创制重大专项（2012ZX09303005-002,2012ZX09303-003）;江苏省重点实验室开放课题（SJ11KF05）;江苏省社会发展项目（BE2011614）

摘　　要：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.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.

关 键 词：额颞叶痴呆 行为障碍 认知障碍 神经心理学测验 磁共振成像 

分 类 号：R749.16[医药卫生—神经病学与精神病学]