Pasquier F, Lebert F, Lavenu I, Petit H
Clinique Neurologique, Hôpital Roger Salengro, Centre Hospitalier et Universitaire, Lille.
Rev Neurol (Paris). 1998 Apr;154(3):217-23.
Frontotemporal dementia (FTD) is poorly recognized clinically. Of the 1,517 patients examined at the Lille Outpatients Memory Clinic data bank (1991-1995), 74 fulfilled the criteria of the Lund and Manchester groups for FTD. They accounted for 5 p, 100 of all patients, 1 for 10 probable or possible Alzheimer's disease. Mean patient age was 63 years, duration of the disease was 5 years, mean Mini Mental State score was 23; 45 p. 100 belonged to the active population. Behavioral disorders occurred before the cognitive decline and remained the major feature. All patients had at least 3 of the following symptoms: self-control impairment, affective disorder, loss of interest and self-neglect. Memory impairment consisted of correct encoding and impaired retrieval processes, without major storage impairment. No patients had spatial disorientation. Language was usually reduced, EEG was normal. Two diagnoses were confirmed by autopsy: both consisted of aspecific frontal and temporal degeneration, 41 p. 100 of the patients were referred by a general practitioner, 30 p. 100 by a psychiatrist, 16 p. 100 by a neurologist, 2 p. 100 by other specialists, and 11 p. 100 following the advice of their relatives. FTD had never been suspected. Alzheimer's disease or non specified degenerative dementia was suspected in 2/3 of patients and a psychiatric disorder in 1/3. With the advent of novel pharmacological agents for the treatment of dementing disorders and for research purposes, the identification and accurate differentiation of FTD from Alzheimer's disease and psychiatric disorder is essential. Therefore, the role of multidisciplinary memory clinic is crucial to differentiate FTD from other degenerative dementias.
额颞叶痴呆(FTD)在临床上很难被识别。在里尔门诊记忆诊所数据库(1991 - 1995年)检查的1517例患者中,74例符合隆德和曼彻斯特小组制定的FTD标准。他们占所有患者的5%,每10例可能或疑似阿尔茨海默病患者中就有1例FTD患者。患者平均年龄为63岁,病程为5年,简易精神状态平均评分为23分;45%属于活跃人群。行为障碍在认知衰退之前出现,并且仍然是主要特征。所有患者至少有以下3种症状:自我控制受损、情感障碍、兴趣丧失和自我忽视。记忆障碍包括正确编码和提取过程受损,但没有严重的存储障碍。没有患者出现空间定向障碍。语言功能通常减退,脑电图正常。尸检确诊了两例:均为非特异性额叶和颞叶变性,41%的患者由全科医生转诊,30%由精神科医生转诊,16%由神经科医生转诊,2%由其他专科医生转诊,11%是根据亲属建议前来就诊。此前从未怀疑过FTD。2/3的患者曾被怀疑患有阿尔茨海默病或未明确的退行性痴呆,1/3的患者曾被怀疑患有精神障碍。随着用于治疗痴呆症和研究目的的新型药物的出现,准确识别FTD并将其与阿尔茨海默病和精神障碍区分开来至关重要。因此,多学科记忆诊所对于区分FTD与其他退行性痴呆起着关键作用。