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。

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

Abstract:

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 ¹⁸F-fluoro-2-deoxy-D-glucose (¹⁸F-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%). ¹⁸F-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 ¹⁸F-FDG PET is helpful for early diagnosis and differential diagnosis.

Key words: Frontotemporal lobar degeneration, Cognition disorders, Olfaction disorders, Neuropsychological tests, Magnetic resonance imaging, Positron-emission tomography, Fluorodeoxyglucose F18