Oinas M, Sulkava R, Polvikoski T, Kalimo H, Paetau A
Department of Pathology, University of Helsinki, Helsinki, Finland.
APMIS. 2007 Jul;115(7):820-7. doi: 10.1111/j.1600-0463.2007.apm_521.x.
Dementia with Lewy bodies (DLB) is a common but underdiagnosed dementing disorder. Its criteria were defined in 1996, and revised in 2005. DLB is characterised neuropathologically by widely distributed cortical Lewy bodies (LBs), usually associated with Alzheimer-type pathology. We have re-evaluated the neuropathology of 55 autopsied patients with clinically diagnosed primary degenerative dementia to determine the frequency of DLB in this cohort, which was originally examined when neither the entity of DLB nor its diagnostic criteria had been defined. We also evaluated how discovery of a new entity affects previous diagnoses. Of the 55 brains, 16 (29%) contained LBs. All 16 originally had a neuropathological diagnosis of Alzheimer's disease (AD). 11 (20%) fulfilled the neuropathological criteria for DLB. Three patients had AD with LBs in the brain stem only, and two patients had LBs in the limbic cortex only. Because the criteria and reliable markers for DLB were not available at the time of the autopsies, the diagnosis of DLB had not been possible. The common co-occurrence of AD-type pathology in DLB makes the clinical diagnosis of DLB problematic even today. This study also raises the question of the relative significances of Lewy-related and AD-type pathologies to the development of dementia.
路易体痴呆（DLB）是一种常见但诊断不足的痴呆症。其诊断标准于1996年确定，并于2005年修订。DLB的神经病理学特征是广泛分布的皮质路易体（LBs），通常与阿尔茨海默病类型的病理改变相关。我们重新评估了55例临床诊断为原发性退行性痴呆的尸检患者的神经病理学，以确定该队列中DLB的发生率，该队列最初检查时DLB这一实体及其诊断标准均未明确。我们还评估了新实体的发现如何影响先前的诊断。在这55个大脑中，16个（29%）含有路易体。最初这16个病例的神经病理学诊断均为阿尔茨海默病（AD）。11个（20%）符合DLB的神经病理学标准。3例患者仅在脑干有AD伴路易体，2例患者仅在边缘皮质有路易体。由于尸检时DLB的标准和可靠标志物尚未可用，当时无法做出DLB的诊断。即使在今天，DLB中常见的AD型病理改变共存也使得DLB的临床诊断存在问题。这项研究还提出了路易体相关病理和AD型病理对痴呆症发展的相对重要性问题。
