Kertesz Andrew
Department of Clinical Neurological Sciences, St. Joseph's Hospital, University of Western Ontario, London, Ontario, Canada.
Can J Neurol Sci. 2006 May;33(2):141-8. doi: 10.1017/s0317167100004893.
Frontotemporal dementia (clinical Pick's disease) is a relatively common, but underdiagnosed degenerative disease in the presenium. Estimated prevalence ranges from 6-12% of dementias. The behavioural, aphasic and extrapyramidal presentations are labeled FTD-behavioural variant, Primary Progressive Aphasia (PPA) and Corticobasal Degeneration/Progressive Supranuclear Palsy (CBD/PSP). The diagnostic features and course of each are described and their overlap in the evolution of the illness is emphasized. The neuropathology ranges from the most common tau negative ubiquitin positive amyotrophic lateral sclerosis (ALS) type inclusions to the tau positive classical Pick bodies and more or less distinct changes of PSP and CBD. The genetics of the relatively frequent tau mutations and the yet unsolved problem of tau negative families are discussed. The tau negative cases tend to be associated with the behavioural presentation and semantic dementia and the tau positive ones with PPA and the CBD/PSP syndrome. However the overlap is too great to split the disease. A glossary to navigate the proliferating terminology is included.
额颞叶痴呆（临床匹克病）是一种相对常见但在早老期易被漏诊的退行性疾病。估计患病率占痴呆症的6% - 12%。其行为、失语和锥体外系表现分别被归类为额颞叶痴呆行为变异型、原发性进行性失语（PPA）以及皮质基底节变性/进行性核上性麻痹（CBD/PSP）。文中描述了每种类型的诊断特征和病程，并强调了它们在疾病演变过程中的重叠情况。神经病理学表现范围广泛，从最常见的tau蛋白阴性、泛素阳性的肌萎缩侧索硬化（ALS）型包涵体，到tau蛋白阳性的典型匹克小体，以及或多或少明显的PSP和CBD改变。文中还讨论了相对常见的tau基因突变的遗传学以及tau蛋白阴性家族尚未解决的问题。tau蛋白阴性病例往往与行为表现和语义性痴呆相关，而tau蛋白阳性病例则与PPA以及CBD/PSP综合征相关。然而，它们之间的重叠程度过大，无法将该疾病进行细分。本文还包含一个术语表，以帮助理解不断增多的专业术语。
