摘要:目的 比较额颞叶痴呆(FTD)与阿尔茨海默病(AD)患者认知功能和神经精神行为的差异以及与痴呆严重程度的关系.方法 选择符合入组条件的FTD患者38例、AD患者46例,分别采用蒙特利尔认知评估量表(MoCA)和简易精神状态检查(MMSE)量表评价认知功能;神经精神量表(NPI)和额叶行为量表(FBI)评价神经精神行为;汉密尔顿抑郁量表21项版本(HAMD-21)评价情绪状态;以及临床痴呆评定量表(CDR)划分痴呆严重程度.结果 与AD患者相比,FTD患者年龄(岁)较小(70.13 ±8.36与66.46±7.04;t =2.124,P=0.037),发病年龄(岁)较旱(68.58±8.51与64.43±6.82;t=2.396,P=0.019),MoCA评分(分)低于AD患者[12.50 (8.00,16.25)与17.00(10.75,21.00);Z=-2.428,P=0.015],而NPI、FBI、FBI-A、FBI-B及HAMD-21分值(分)高于AD患者[NPI:15.00(7.00,25.50)与9.50(4.00,17.75),Z=-2.251,P=0.024;FBI:21.00(13.00,27.00)与16.00(10.75,23.00),Z=-2.159,P =0.031; FBI-A:13.00(8.00,16.00)与9.00 (6.00,12.00),Z=-2.159,P=0.041; FBI-B:9.00 (7.00,14.00)与7.00(3.00,11.00),Z=-2.051,P=0.040;HAMD-21:7.00(2.75,14.00)与5.00(3.00,8.00),Z=-2.061,P=0.039];亚项分析显示:FTD患者在执行功能(Z=-2.140,P=0.032)、语言(Z=-3.357,P=0.001)、抽象能力(Z=-2.498,P=0.012)和延迟回忆(Z=-4.317,P=0.000)等认知方面差于AD患者,AD患者的记忆力(Z=-1.999,P=0.046)和定向力(Z=-2.941,P=0.003)较FTD患者损害明显;FTD患者在激越(Z=-3.255,P=0.001)、脱抑制(Z=-3.093,P=0.002)和易激惹(Z=-2.214,P=0.027)等神经精神行为障碍较AD患者严重;NPI(r =0.279,P=0.010)、FBI(r=0.353,P=0.001)、FBI-A(r =0.386,P=0.000)和FBI-B(r=0.273,P=0.012)与CDR评分呈正相关,MoCA评分与CDR评分呈负相关(r=-0.760,P=0.000);两组患者的MoCA和NPI各亚项分值随着痴呆程度加重发生相应变化.结论 FTD和AD患者在认知功能和精神行为症状方面存在差异,FTD患者执行功能、语言、抽象能力和延迟回忆较差,而AD患者记忆力、定向力较差.随着病情的发展,FTD患者逐渐出现定向力障碍,AD患者的认知功能障碍几乎波及各个领域.FTD患者激越、脱抑制、易激惹行为多见,AD患者病程晚期出现抑郁.临床工作中对痴呆患者的认知功能和精神行为症状的动态观察有助于FTD和AD的鉴别.%Objective The purpose of this study was to investigate the differences of cognitive impairment and neuropsychiatric behavior disturbances between Alzheimer's disease (AD) and frontotemporal dementia (FTD) patients,as well as their relationships with dementia severity.Methods A total of 38 FTD patients and 46 AD patients were recruited in this study.The Montreal Cognitive Assessment (MoCA) and Mini-Mental State Examination (MMSE) were used to evaluate the degree of cognitive impairments.The Neuropsychiatric Inventory Brief Questionnaire Form (NPI) and Frontal Behavioral Inventory (FBI) were used to measure behavioral disturbances.The 21-items Hamilton Depression Rating Scale (HAMD-21) was used to evaluate the mental or emotional state of patients.Clinical dementia rating scale (CDR) was used to divide the dementia severity.Results FTD patients were younger ((70.13 ± 8.36) years vs (66.46 ± 7.04) years,t =2.124,P =0.037),earlier at age of onset ((68.58 ± 8.51) years vs (64.43 ± 6.82) years,t =2.396,P =0.019),with lower MoCA scores (12.50 (8.00,16.25) vs 17.00(10.75,21.00),Z=-2.428,P=0.015),higher NPI (15.00(7.00,25.50)vs 9.50(4.00,17.75),Z=-2.251,P=0.024),FBI (21.00(13.00,27.00)vs 16.00(10.75,23.00),Z=-2.159,P=0.031),FBI-A (13.00 (8.00,16.00)vs 9.00(6.00,12.00) Z=-2.159,P=0.041),FBI-B (9.00(7.00,14.00) vs 7.00(3.00,11.00),Z=-2.051,P=0.040) and HAMD-21 scores (7.00(2.75,14.00) vs 5.00 (3.00,8.00),Z =-2.061,P =0.039).A detail analysis of different cognitive domains showed the executive functions (Z =-2.140,P =0.032),language (Z =-3.357,P =0.001),abstraction (Z =-2.498,P =0.012) and delayed recall (Z =-4.317,P =0.000) of the MoCA scale were lower in FTD patients than that in AD patients,while AD patients had lower scores in memory (Z =-1.999,P =0.046) and orientation (Z =-2.941,P =0.003) of the MMSE scale.Within the subscale scores of the NPI,the agitation (Z =-3.255,P =0.001),disinhibition (Z =-3.093,P =0.002) and irritability (Z =-2.214,P =0.027) scores were higher in FTD patients than in AD patients.The total scores of NPI (r=0.279,P=0.010),FBI (r =0.353,P=0.001),FBI-A (r=0.386,P=0.000) and FBI-B (r =0.273,P =0.012) were positively correlated with the CDR scores,whereas MoCA scores were negatively correlated with the CDR scores (r =-0.760,P =0.000).The subscale scores on MoCA and NPI areas changed corresponding with dementia severity in both groups.Conclusions The cognitive function,behavioral and psychological symptoms between FTD and AD patients are different.FTD patients have poorer executive function,language,abstraction and delayed recall ability,whereas AD patients perform worse in memory and orientation.With the progression of the disease,FTD patients gradually emerged disorientation,while the cognitive impairment in AD patients almost affected all the areas.FTD patients are more likely to have agitation,disinhibition and irritability behavior,and AD patients are more likely to have depression in the late stage.Dynamic evaluation of the cognitive function,behavioral and psychological symptoms in clinical practice can help to distinguish FTD and AD.