Jackson M, Lowe J
Department of Neurology, University of Nottingham Medical School, UK.
Acta Neuropathol. 1996;91(2):127-34. doi: 10.1007/s004010050403.
The clinical features and recent developments in the neuropathology of frontotemporal dementia are reviewed. The five main neurodegenerative disorders that underlie the clinical syndrome of frontotemporal dementia are distinguished using immunohistochemistry with antisera to ubiquitin and tau proteins. Motor neuron disease-type dementia is characterised by ubiquitin-immunoreactive intraneuronal inclusions in cortical layer II and the hippocampal dentate granule cells. A diagnosis of Alzheimer's disease changes is based upon the presence of neurofibrillary tangles, which immunostain with antibodies to tau and ubiquitin, and many associated neuritic plaques. Corticobasal degeneration is diagnosed by the presence of tau-immunoreactive, but ubiquitin-non-reactive intraneuronal inclusions in cortical layer II and the substantia nigra. Pick's disease is restricted to cases with tau- and ubiquitin-immunoreactive spherical cortical intraneuronal inclusions (Pick bodies), best seen in the hippocampal dentate gyrus and frontotemporal cortex. Dementia of frontal type is the preferred term for cases in which no intraneuronal inclusions are seen with antisera to tau and ubiquitin. A practical approach to the pathological diagnosis of frontotemporal dementia and the differential diagnosis of the five disorders using immunohistochemical studies is provided.
本文综述了额颞叶痴呆的临床特征及神经病理学的最新进展。利用针对泛素和tau蛋白的抗血清进行免疫组织化学，区分了构成额颞叶痴呆临床综合征基础的五种主要神经退行性疾病。运动神经元病型痴呆的特征是皮质第二层和海马齿状颗粒细胞中存在泛素免疫反应性神经元内包涵体。阿尔茨海默病改变的诊断基于神经原纤维缠结的存在，其用抗tau和泛素抗体进行免疫染色，以及许多相关的神经炎性斑块。皮质基底节变性通过皮质第二层和黑质中存在tau免疫反应性但泛素非反应性的神经元内包涵体来诊断。匹克病仅限于具有tau和泛素免疫反应性球形皮质神经元内包涵体（匹克小体）的病例，在海马齿状回和额颞叶皮质中最易观察到。额型痴呆是指用抗tau和泛素抗血清未观察到神经元内包涵体的病例的首选术语。本文提供了一种利用免疫组织化学研究对额颞叶痴呆进行病理诊断及对这五种疾病进行鉴别诊断的实用方法。
