Hughes A J
Department of Neurology, Austin and Repatriation Medical Centre (Repatriation Campus), Heidelberg West, Vic., Australia.
Eur Neurol. 1997;38 Suppl 2:13-20. doi: 10.1159/000113471.
Clinicopathological studies have shown that the accuracy of a clinical diagnosis of Parkinson's disease (PD) is less than 80%. More selected diagnostic criteria can increase the proportion of true PD cases identified to more than 90%. However, by using these criteria almost a third of pathologically confirmed cases may be rejected. Awareness of the high misdiagnosis rate and refinements in the clinical diagnostic criteria for other conditions that may present with parkinsonism seem to have led to an improvement in the accuracy of diagnosis to 84%. Cortical Lewy bodies, often in small numbers, are seen in almost all patients with PD. This has prompted intense efforts to clarify both the clinical and pathological criteria for diffuse Lewy body disease. The nomenclature of conditions with plentiful numbers of Lewy bodies in the neocortex and the relevance of associated pathology has become confusing and the subject of intense nosological debate. A recent consensus paper has suggested clinical and pathological diagnostic criteria for what has been termed 'dementia with Lewy bodies'. This new term awaits general acceptance while the proposed diagnostic criteria remain to be validated. The neuropathological substrate of dementia in PD continues to receive a lot of attention with suggestions that additive deficits from pathology in both cortical and subcortical structures probably explain most of the dementia seen in PD.
临床病理研究表明，帕金森病（PD）临床诊断的准确率低于80%。更严格的诊断标准可使确诊的真正PD病例比例增至90%以上。然而，采用这些标准时，几乎三分之一经病理证实的病例可能会被排除。对高误诊率的认识以及对可能表现为帕金森综合征的其他疾病临床诊断标准的完善，似乎已使诊断准确率提高到了84%。几乎所有PD患者都可见到数量通常较少的皮质路易小体。这促使人们努力去明确弥漫性路易体病的临床和病理标准。新皮质中存在大量路易小体的疾病命名以及相关病理学的关联性已变得令人困惑，成为了激烈的疾病分类学争论的主题。最近的一篇共识论文提出了针对所谓“路易体痴呆”的临床和病理诊断标准。在这些拟议的诊断标准得到验证之前，这个新术语尚有待普遍接受。PD中痴呆的神经病理学基础一直备受关注，有观点认为，皮质和皮质下结构的病理学累加缺陷可能解释了PD中所见的大部分痴呆。
