Pezzoli G, Canesi M, Galli C
Department of Neuroscience, Parkinson Institute, Istituti Clinici di Perfezionamento, Via Bignami, 1, Milano 20126, Italy.
Sleep Med. 2004 Mar;5(2):181-7. doi: 10.1016/j.sleep.2003.10.009.
Recent molecular biology research on neurodegenerative diseases, including parkinsonisms, has identified mutations in the genes that code for the proteins alpha-synuclein and tau, which have been used to classify them into synucleinopathies and tauopathies. The synucleinopathies include, besides the most common and well studied Parkinson's disease (PD), dementia with Lewy bodies, which accounts for approximately 20% of all cases of dementia in the elderly, and multiple system atrophy, whereas the tauopathies include rare and rapidly progressive syndromes, such as progressive supranuclear palsy and corticobasal degeneration. Data we collected at our center in over 2900 parkinsonian patients show that PD accounts for no more than 70% of parkinsonisms. The various syndromes have many features in common that make the differential diagnosis difficult in the early stages of disease. Our data are consistent with the findings reported in the international literature and provide additional information useful for differential diagnosis.
最近针对包括帕金森症在内的神经退行性疾病开展的分子生物学研究,已确定了编码α-突触核蛋白和tau蛋白的基因突变,这些基因突变已被用于将神经退行性疾病分为突触核蛋白病和tau蛋白病。除了最常见且研究充分的帕金森病(PD)外,突触核蛋白病还包括路易体痴呆(约占老年痴呆病例总数的20%)和多系统萎缩;而tau蛋白病则包括罕见且进展迅速的综合征,如进行性核上性麻痹和皮质基底节变性。我们中心收集的2900多名帕金森症患者的数据显示,PD在帕金森症中所占比例不超过70%。各种综合征有许多共同特征,这使得在疾病早期进行鉴别诊断很困难。我们的数据与国际文献报道的结果一致,并为鉴别诊断提供了额外的有用信息。