Gigi A, Pirrotta R, Kelley-Puskas M, Lazignac C, Damsa C
Centre Hospitalier Luxembourg, 4, rue Barblé, L-1210 Luxembourg.
Encephale. 2006 Oct;32(5 Pt 1):775-80. doi: 10.1016/s0013-7006(06)76230-3.
The diagnosis of fronto-temporal dementia (FTD) represents a challenge for the psychiatrist, especially since this insidious pathology partly mimics other psychiatric diseases.
CASE-REPORT: We present a clinical case that illustrates the difficulty of FTD diagnosis particularly well. A 32 year-old woman without previous medical history presented with psychomotor agitation, logorrhea and flight of ideas. The criteria for bipolar disorder according to DSM IV were met and the patient was referred to an outpatient clinic where a mood stabilizer was initiated (lithium, 400 mg/day). An in-depth interview with her husband revealed mild but progressive modification of her personality and behavior over the course of two years. She showed signs of mild fatigue and irritability that evolved into a loss of interest for both leisure and domestic activities. In addition, she showed increasing erratic behavior and emergence of frequent episodes of verbal abuse. After the birth of her second child, the patient's clinical state worsened with the appearance of uninhibited behavior, loss of personal hygiene, sleep disturbances and nightmares. The patient was forced to stop her work as a cleaner, a steady employment that she had maintained for 10 years. The hypomanic state worsened and psychotic symptoms such as delusions and echolalia appeared within a few weeks. These events culminated in a first hospitalization in a psychiatric unit. We evoked both diagnoses of schizoaffective disorder and psychotic disorder not otherwise specified. In the following months, we conducted neurological examinations on account of the progressive deterioration of her cognitive functions. Neuroradiological results (CT scan with contrast agents, MRI, cerebral scintigraphy) coupled with her clinical evolution (neurological examination and neuropsychological testing) permitted diagnosis of fronto-temporal dementia.
Fronto-temporal dementia usually presents itself as an autosomal dominant disease in 89% of reported cases, with an insidious onset associated with thymic symptoms and behavioral disturbances. The first consultation often concludes with a suspicion of a psychiatric disorder in 33% of the cases (unipolar and bipolar depressive disorders, psychotic disorders, alcohol dependence). The clinical description of such a heterogenic and neuro-psychiatric disorder should be widely disseminated, so that psychiatrists can distinguish early symptoms and diminish the risk of misdiagnosis of FDT.
Such case reports emphasize the importance for psychiatrists to be aware of the clinical prodromal FDT symptoms, particularly since the neuro-imaging data of dementia are often delayed.
额颞叶痴呆(FTD)的诊断对精神科医生来说是一项挑战,尤其是因为这种隐匿性疾病部分类似于其他精神疾病。
我们呈现一个临床病例,该病例很好地说明了FTD诊断的困难。一名32岁、既往无病史的女性出现精神运动性激越、多语症和思维奔逸。符合DSM-IV中双相情感障碍的标准,该患者被转诊至门诊并开始使用心境稳定剂(锂盐,400毫克/天)。对其丈夫的深入访谈显示,在两年时间里她的人格和行为有轻微但渐进性的改变。她表现出轻度疲劳和易怒的迹象,逐渐发展为对休闲和家务活动都失去兴趣。此外,她的行为越来越不稳定,频繁出现言语辱骂。在她第二个孩子出生后,患者的临床状态恶化,出现行为无节制、个人卫生习惯丧失、睡眠障碍和噩梦。患者被迫辞去她做了10年的清洁工工作。轻躁狂状态恶化,几周内出现妄想和模仿言语等精神病性症状。这些情况最终导致她首次住进精神科病房。我们考虑了精神分裂症性障碍和未另行特指的精神病性障碍这两种诊断。在接下来的几个月里,由于她认知功能的逐渐恶化,我们进行了神经系统检查。神经放射学结果(增强CT扫描、MRI、脑闪烁显像)以及她的临床进展(神经系统检查和神经心理学测试)确诊为额颞叶痴呆。
在89%的报告病例中,额颞叶痴呆通常表现为常染色体显性疾病,起病隐匿,伴有精神症状和行为障碍。在33%的病例中,首次就诊时通常会怀疑患有精神疾病(单相和双相抑郁障碍、精神病性障碍、酒精依赖)。这种异质性神经精神疾病的临床描述应广泛传播,以便精神科医生能够识别早期症状并降低FTD误诊的风险。
此类病例报告强调了精神科医生了解FTD临床前驱症状的重要性,特别是因为痴呆的神经影像学数据往往延迟出现。