Ichikawa Hiroo, Kawamura Mitsuru
Department of Neurology, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan.
Brain Nerve. 2009 Nov;61(11):1227-35.
Frontotemporal dementia (FTD)-a common clinical manifestation of frontotemporal lobar degeneration (FTLD)--is characterized by alterations in personality and social conduct. Its symptoms include inertia, loss of volition, social disinhibition, and distractibility, with relative preservation of memory and visuospatial function. We present the typical case of patient with typical clinical symptoms including "going my way" behavior, inactivity, lack of awareness of illness, stereotypic behavior, perseveration, and environmental dependency syndrome. These clinical symptoms can be interpreted on the basis of extent of damage to the frontal lobes and the interaction between the frontal lobes and other neural systems such as the posterior association cortices, basal ganglia or limbic systems. We also address several complex clinical issues, including the relationship between clinical manifestations and pathological findings, underestimation of FTD in patients with amyotrophic lateral sclerosis (ALS) and/or motor neuron disease (MND), and impairment in a single cognitive domain such as isolated agraphia in ALS/MND and FTD. To address these problems, it is essential to observe the clinical symptoms in patients with FTD and ALS/MND in detail and to compare clinical characteristics with pathological findings. It is also critical to develop clinical tests that minimize the impact of speech and motor dysfunction on performance, particularly on the basis of a longitudinal analysis.
额颞叶痴呆(FTD)——额颞叶变性(FTLD)的一种常见临床表现——以人格和社会行为改变为特征。其症状包括惰性、意志丧失、社会行为脱抑制和注意力分散,而记忆和视觉空间功能相对保留。我们呈现了一例具有典型临床症状的患者,这些症状包括“我行我素”行为、活动减少、无疾病意识、刻板行为、持续言语和环境依赖综合征。这些临床症状可以根据额叶受损程度以及额叶与其他神经系统(如后联合皮质、基底神经节或边缘系统)之间的相互作用来解释。我们还讨论了几个复杂的临床问题,包括临床表现与病理结果之间的关系、肌萎缩侧索硬化(ALS)和/或运动神经元病(MND)患者中FTD的漏诊,以及单一认知领域的损害,如ALS/MND和FTD中的孤立性失写症。为了解决这些问题,详细观察FTD和ALS/MND患者的临床症状并将临床特征与病理结果进行比较至关重要。开发能够尽量减少言语和运动功能障碍对测试表现影响的临床测试也很关键,尤其是基于纵向分析的测试。