额颞叶变性患者认知功能和嗅觉功能特点  

Cognitive and olfactory function profiles of frontotemporal lobar degeneration

作　　者：豆玉超 李雨晴 纪勇[1] 李攀[1] 周玉颖[1] DOU Yu-chao;LI Yu-qing;JI Yong;LI Pan;ZHOU Yu-ying(Department of Neurology,Tianjin Huanhu Hospital,Tianjin Key Laboratory of Cerebral Vascular and Neurodegenerative Diseases,Tianjin 300350,China)

机构地区：[1]天津市环湖医院神经内科,天津市脑血管与神经变性重点实验室,300350

出　　处：《中国现代神经疾病杂志》2021年第11期976-981,共6页Chinese Journal of Contemporary Neurology and Neurosurgery

基　　金：国家自然科学基金青年科学基金资助项目(项目编号:81801076)。

摘　　要：目的总结额颞叶变性患者的认知功能和嗅觉功能特点。方法纳入2014年7月至2017年4月天津市环湖医院收治的52例额颞叶变性患者,采用简易智能状态检查量表(MMSE)和蒙特利尔认知评价量表(MoCA)评估认知功能、神经精神科问卷(NPI)评估神经精神行为,T&T嗅觉计定量检查(29例)和嗅觉fMRI(6例)评估嗅觉功能,18F-脱氧葡萄糖(18F-FDG)PET(6例)评估脑组织葡萄糖代谢。结果 52例患者认知功能障碍主要表现为执行功能障碍占71.15%(37/52),脱抑制占59.62%(31/52),淡漠占44.23%(23/52),缺乏同情心占40.38%(21/52),记忆力减退占32.69%(17/52),口欲亢进占25%(13/52),语言障碍占3.85%(2/52)。MMSE评分为18(4,20),MoCA评分为11(3,15)且视空间与执行功能、注意力、语言功能、抽象力、延迟回忆、定向力评分均低于正常参考值;NPI评分为24(7,39),主要表现为情感淡漠、易激惹、激越、情绪高涨/欣快。根据T&T嗅觉计定量检查识别域,嗅觉正常1例(3.45%),轻度嗅觉减退12例(41.38%),中度嗅觉减退9例(31.03%),重度嗅觉减退5例(17.24%),嗅觉丧失2例(6.90%);嗅觉fMRI显示,由低浓度至高浓度(0.10%、0.33%、1.00%)依次进行气体刺激时,患者对气味产生适应。18F-FDG PET显示,对称性(2例)或非对称性(4例)额叶和(或)颞叶为主的葡萄糖低代谢。结论额颞叶变性患者以执行功能障碍和神经精神行为异常多见,记忆障碍出现较晚;亦可出现嗅觉功能异常,但是嗅觉适应能力仍存在。神经心理学测验联合18F-FDG PET有助于早期诊断与鉴别诊断。Objective To describe the cognitive and olfactory function profiles of frontotemporal lobar degeneration(FTLD).Methods A total of 52 FTLD patients admitted to Tianjin Huanhu Hospital from July 2014 to April 2017 were enrolled.Cognitive function was assessed by the Mini-Mental State Examination(MMSE) and Montreal Cognitive Assessment(MoCA),and neuropsychiatric behavior was assessed by Neuropsychiatric Inventory(NPI).Quantitative olfactory function was assessed by T&T olfactory meter and olfactory fMRI,and changes in glucose metabolism in brain tissues were assessed by;Ffluoro-2-deoxy-D-glucose(;F-PDG) PET.Results The cognitive dysfunction of 52 patients was mainly manifested as executive dysfunction(71.15%,37/52),disinhibition(59.62%,31/52),indifference(44.23%,23/52),lack of empathy(40.38%,21/52),memory loss(32.69%,17/52),hyperstomia(25%,13/52) and language disorder(3.85%,2/52).MMSE score was 18(4,20),MoCA score was 11(3,15),and visuospatial and executive function,attention,language function,abstraction,delayed recall,and orientation were all lower than normal reference values.NPI score was 24(7,39),and the main manifestations were apathetic,irritable,excited,and high/euphoric.According to the T&T olfactory meter recognition domain,one case(3.45%) had normal sense of smell,12 cases(41.38%) had mild hyposmia,9 cases(31.03%) had moderate hyposmia,5 cases(17.24%) had severe hyposmia,and 2 cases(6.90%) had anosmia.Olfactory fMRI showed that patients developed adaptation to odor when olfactory stimulation with gas concentration was successively from low to high(0.10%,0.33%,1.00%).;F-FDG PET showed symmetric(2 cases) or asymmetric(4 cases) predominance of the frontal and/or temporal lobes.Conclusions In FTLD patients,executive dysfunction and neuropsychiatric behavior abnormalities were more common,while memory impairment appeared later.Abnormal olfactory function may also occur,but olfactory adaptation still exists.Neuropsychological test combined with;F-FDG PET is helpful for early diagnosis and differential diagnosis

关 键 词：额颞叶变性 认知障碍 嗅觉障碍 神经心理学测验 磁共振成像 正电子发射断层显像术 氟脱氧葡萄糖F18 

分 类 号：R749.1[医药卫生—神经病学与精神病学]