Psychiatry, Osaka University Graduate School of Medicine, Suita, Japan.
Psychiatry Clin Neurosci. 2022 Aug;76(8):351-360. doi: 10.1111/pcn.13375. Epub 2022 Jun 2.
Frontotemporal dementia is a neurodegenerative disease characterized by focal degeneration of the frontal and temporal lobes, clinically presenting with disinhibited behavior, personality changes, progressive non-fluent aphasia and/or impaired semantic memory. Research progress has been made in re-organizing the clinical concept of frontotemporal dementia and neuropathological classification based on multiple accumulating proteins. Alongside this progress a list of genetic mutations or variants that are causative or increase the risk of frontotemporal dementia have been identified and some of these gene products are extensively studied. However, there are still a lot of points that need to be overcome, including lack of specific diagnostic biomarker which enable antemortem diagnosis of underlying neurodegenerative process, and lack of disease modifying therapy which could prevent disease progression. Early and precise diagnosis of frontotemporal dementia is urgently required. In this context, how to define prodromal frontotemporal dementia and early differential diagnosis from primary psychiatric disorders are also important issues. In this review we will summarize and discuss current understanding of biological basis and psychiatric symptoms in frontotemporal dementia.
额颞叶痴呆是一种神经退行性疾病，其特征是额颞叶的局灶性变性，临床上表现为行为抑制障碍、人格改变、进行性非流利性失语症和/或语义记忆受损。基于多种积累蛋白，在重新组织额颞叶痴呆的临床概念和神经病理学分类方面取得了研究进展。随着这一进展，已确定了一些导致或增加额颞叶痴呆风险的遗传突变或变异，其中一些基因产物正在广泛研究。然而，仍有许多问题需要克服，包括缺乏特异性诊断生物标志物，无法对潜在神经退行性过程进行生前诊断，以及缺乏能够阻止疾病进展的疾病修饰疗法。因此，急需对额颞叶痴呆进行早期和准确的诊断。在这种情况下，如何定义前驱期额颞叶痴呆以及与原发性精神障碍的早期鉴别诊断也是重要的问题。在本文综述中，我们将总结和讨论目前对额颞叶痴呆的生物学基础和精神症状的认识。
