Abstract:

Background  Although the early behavioral symptoms of behavioral variant frontotemporal dementia (bvFTD) are prominent, early diagnosis for bvFTD is difficult due to confusion with other mental disorders, and lack of sensitivity and specificity of diagnostic criteria, etc. In this paper, we summarized the important reviews in recent years and analyzed the clinical characteristics of bvFTD patients to improve the detection of early symptoms in bvFTD.  Methods Twenty-three possible or probable bvFTD patients were diagnosed according to International Behavioral Variant Frontotemporal Dementia Criteria Consortium (FTDC). Self-designed questionnaires designed by Shanghai Mental Health Center were used to collect sociodemographic data and general information of patients. Their clinical characteristics were summarized, including abnormal behaviors, cognitive impairment, psychotic symptoms and other symptoms. Mini-Mental State Examination (MMSE), Activities of Daily Living (ADL) and Clinical Dementia Rating Scale (CDR) were used to make neuropsychological tests and compare with similar overseas studies (control group, N = 66).  Results  Eleven male patients and 12 female patients were
included in our study. Compared with control group, the average age of onset [(50.83 ± 11.55) years vs. (57.00 ± 10.00) years; t = 3.863, P = 0.000] and average age of diagnosis [(53.22 ± 11.55) years vs. (61.00 ± 9.00) years; t = 13.423, P = 0.000] of bvFTD patients were smaller. The study showed that bvFTD patients had more apathy or indolence [95.65% (22/23) vs. 65.15% (43/66); χ² = 8.057, P = 0.005], loss of sympathy or empathy [95.65% (22/23) vs. 33.33% (22/66); χ² = 26.499, P = 0.000], while patients in control group showed more derepression behavior [98.48% (65/66) vs. 52.17% (12/23); χ² = 27.514, P = 0.000] and continuous, stiff, obsessive and/or ritualized behavior [95.45% (63/66) vs. 30.43% (7/23); adjusted χ² = 39.159, P = 0.000]. For cognitive impairment, bvFTD patients presented apraxia [69.56% (16/23) vs. 22.73% (15/66); χ² = 16.484, P = 0.000] and executive dysfunction [82.61% (19/23) vs. 59.09% (39/66); χ² = 4.156, P = 0.041]. In psychotic symptoms, patients in control group showed hallucination and delusion [33.33% (22/66) vs. 4.35% (1/23); χ² = 7.477, P = 0.006].  Conclusions  While the behavioral symptoms of bvFTD patients are varied, memory disorder can be one of the primal symptoms. Compared with similar foreign studies, the different composition of behavior symptoms of our bvFTD patients may be caused by cultural differences among evaluators in the understanding of some clinical symptoms in patients.

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摘要：

研究背景 行为异常型额颞叶痴呆（bvFTD）患者早期异常行为症状突出，但是由于易与其他精神病混淆、诊断标准敏感性和特异性较低，临床早期诊断困难。本文总结行为异常型额颞叶痴呆患者早期临床特点并结合近年文献进行比较，以期提高早期诊断与鉴别诊断水平。方法 共23 例经行为异常型额颞叶痴呆国际标准联盟诊断标准诊断的很可能的和可能的行为异常型额颞叶痴呆患者（bvFTD 组），采用上海市精神卫生中心自行编制的调查表收集社会人口学资料和临床资料，总结临床特点（包括异常行为、认知功能障碍、精神病性症状、其他症状），采用简易智能状态检查量表、日常生活活动能力量表和临床痴呆评价量表进行神经心理学测验，并与国外相似研究（对照组，66 例）进行比较。结果 bvFTD 组患者发病年龄［（50.83 ± 11.55）岁对（57.00 ± 10.00）岁；t = 3.863，P = 0.000］和明确诊断年龄［（53.22 ± 11.55）岁对（61.00 ± 9.00）岁；t = 13.423，P = 0.000］更小。异常行为方面，bvFTD 组患者更多表现为淡漠或懒惰［95.65%（22/23）对65.15%（43/66）；χ² = 8.057，P = 0.005］，同情和（或）同理心缺失［95.65%（22/23）对33.33%（22/66）；χ² = 26.499，P = 0.000］；对照组患者更多表现为脱抑制行为［98.48%（65/66）对52.17%（12/23）；χ² = 27.514，P = 0.000］，持续、刻板、强迫性和（或）仪式化行为［95.45%（63/66）对30.43%（7/23）；校正χ² = 39.159，P = 0.000］；认知功能障碍方面，bvFTD 组患者更多表现为失用［69.56%（16/23）对22.73%（15/66）；χ² = 16.484，P = 0.000］和执行功能障碍［82.61%（19/23）对59.09%（39/66）；χ² = 4.156，P = 0.041］；精神病性症状方面，对照组患者更多表现为幻觉妄想［33.33%（22/66）对4.35%（1/23）；χ² = 7.477，P = 0.006］。结论 行为异常型额颞叶痴呆患者临床症状多样，记忆障碍可能是突出症状之一。与国外相似研究相比，临床症状的构成比不同可能是由于评价者对部分症状的理解存在文化差异。

关键词: 痴呆, 额叶, 颞叶, 神经心理学测验