摘要
目的探讨额颞叶痴呆(FTD)患者的临床及影像学特点。方法回顾性分析56例诊断为很可能FTD患者的临床资料。结果 56例FTD患者中行为变异型额颞叶痴呆(bv FTD) 39例(69. 6%),进行性非流利性失语(PNFA) 13例(23. 2%),语义性痴呆(SD) 4例(7. 1%)。本组患者首次诊断符合率为53. 6%(30/56)。FTD患者首发症状主要包括精神行为异常41例(73. 2%),记忆力下降39例(69. 6%),执行功能障碍36例(64. 3%),语言障碍12例(21. 4%)。随着病程进展,患者精神行为异常、记忆力下降、语言障碍等症状重叠出现或先后出现。根据出现频率高低依次为:记忆力下降39例(69. 6%),执行功能障碍36例(64. 3%),行为脱抑制29例(51. 8%),口欲亢进或饮食习惯改变26例(46. 4%),淡漠25例(44. 6%),兴趣爱好减少25例(44. 6%),嗅觉障碍24例(42. 9%),语言障碍23例(41. 1%),人格改变21例(37. 5%),睡眠障碍20例(35. 7%),收藏行为16例(28. 6%),强迫行为11例(19. 6%),同情心下降10例(17. 9%),刻板动作10例(17. 9%),小便次数增多或小便失禁10例(17. 9%),迷路8例(14. 3%),夜间徘徊8例(14. 3%),焦虑6例(10. 7%),幻觉5例(8. 9%),抑郁4例(7. 1%),妄想4例(7. 1%),坐立不安4例(7. 1%),帕金森样症状4例(7. 1%),肌肉萎缩1例(1. 8%),吞咽困难1例(1. 8%)。45例FTD患者完成MMSE检查,得分在5-27分之间,平均(17. 49±5. 76)分,bv FTD、PNFA、SD患者的MMSE得分均无统计学差异(P> 0. 05)。MRI检查表现为局限性额叶、颞叶前部萎缩,多为不对称性,萎缩程度左侧大于右侧,伴或不伴不同程度海马萎缩。4例病程超过8年的患者额叶或颞叶明显萎缩呈"树枝状"。结论 FTD患者起病年龄早,首诊符合率低。临床症状复杂多样,首发症状主要为精神行为异常、执行功能障碍、记忆力下降、语言功能障碍等;典型的影像学改变为额叶或(和)颞叶萎缩,左侧更为明显,伴或不伴海马萎缩。
Objective To investigate the clinical and neuroimaging features of frontotemporal dementia (FTD). Methods Retrospective analysis of clinical and neuroimaging features of 56 patients diagnosed with probable FTD. Results Among the 56 FTD patients,39 cases (69.6%)were diagnosed as behavioral variant of frontotemporal dementia (bvFTD),13 cases (23. 2%)were diagnosed as progressive non-fluent aphasia (PNFA ),4 cases (7.1%)were diagnosed as semantic dementia (SD). the first diagnosis coincidence rate of this group of FTD patients was 53.6%(30/56 ). The first symptoms included abnormal mental behavior in 41 cases (73.2%), memory decline in 39 cases (69.6%),executive dysfunction in 36 cases (64.3%)and language disorders in 12 cases (21.4%).As the course of disease progresses,symptoms such as mental and behavioral disorders,memory loss,language disorders overlap or appear successively. According to the frequency of occurrence,they are:memory decline in 39 cases (69.6%),executive dysfunction in 36 cases (64.3%),behavioral de-suppression in 29 cases (51.8%),hyperphagia or changes in eating habits in 26 cases (46.4%),indifference in 25 cases (44.6%), hobbies decreased in 25 cases (44.6%),personality disorder in 24 cases (42.9%),language disorders in 23 case (41.1%),olfactory decli or loss in 21 cases (37.5%),sleep disorders in 20 cases (35.7%),collection behavior in 16 cases (28. 6%),forced behavior in 11 cases (19. 6%),compassion decreased in 10 cases (17.9%), stereotyped action in 10 cases (17.9%),urine frequency increased or incontinence in 10 cases (17.9%),getting lost in 8 cases (14.3%),nocturnal wandering in 8 cases (14.3%),anxiety in 6 cases (10.7%),hallucination in 5 cases (8.9%),depression in 4 cases (7.1%),delusion in 4 cases (7.1%),restlessness in 4 cases (7.1%), Parkinson's symptoms in 4 cases (7.1%)and muscle atrophy in 1 case (1.8%),dysphagia was found in 1 cases (1.8%).The MMSE scores of 45 patients with FTD ranged from 5 to 27,with an average of (17.49 ± 5.76). There was no significant difference in MMSE sco
作者
胡秀秀
董靖德
刘忆
郑慧芬
刘斌
靳凌
HU Xiu-xiu;DONG Jing-de;LIU Yi(Department of Geriatrics,Brain Hospital Affiliated to Nanjing Medical University,Nanjing 210029,China)
出处
《临床神经病学杂志》
CAS
2018年第6期436-440,共5页
Journal of Clinical Neurology
关键词
额颞叶痴呆
精神行为异常
语言障碍
影像学特点
frontotemporal dementia
abnormal mental behavior
language dysfunction
imaging features
