Litvan I, Hauw J J, Bartko J J, Lantos P L, Daniel S E, Horoupian D S, McKee A, Dickson D, Bancher C, Tabaton M, Jellinger K, Anderson D W
Neuroepidemiology Branch, National Institute of Neurological Disorders and Stroke, Bethesda, MD 20892, USA.
J Neuropathol Exp Neurol. 1996 Jan;55(1):97-105. doi: 10.1097/00005072-199601000-00010.
We investigated the validity and reliability of diagnoses made by eight neuropathologists who used the preliminary NINDS neuropathologic diagnostic criteria for progressive supranuclear palsy (PSP) and related disorders. The specific disorders were typical, atypical, and combined PSP, postencephalitic parkinsonism, corticobasal ganglionic degeneration, and Pick's disease. These disorders were chosen because of the difficulties in their neuropathologic differentiation. We assessed validity by measuring sensitivity and positive predictive value. Reliability was evaluated by measuring pairwise and group agreement. From a total of 62 histologic cases, each neuropathologist independently classified 16 to 19 cases for the pairwise analysis and 5 to 6 cases for the group analysis. The neuropathologists were unaware of the study design, unfamiliar with the assigned cases, and initially had no clinical information about the cases. Our results showed that with routine sampling and staining methods, neuropathologic examination alone was not fully adequate for differentiating the disorders. The main difficulties were discriminating the subtypes of PSP and separating postencephalitic parkinsonism from PSP. Corticobasal ganglionic degeneration and Pick's disease were less difficult to distinguish from PSP. The addition of minimal clinical information contributed to the accuracy of the diagnosis. On the basis of results obtained, we propose clinicopathologic diagnostic criteria to improve on the NINDS criteria.
我们调查了八位神经病理学家根据美国国立神经疾病与中风研究所（NINDS）初步的进行性核上性麻痹（PSP）及相关疾病的神经病理学诊断标准所做出诊断的有效性和可靠性。具体疾病包括典型、非典型及混合型PSP、脑炎后帕金森综合征、皮质基底节变性和匹克病。选择这些疾病是因为它们在神经病理学鉴别上存在困难。我们通过测量敏感性和阳性预测值来评估有效性。通过测量两两之间及组间一致性来评估可靠性。在总共62例组织学病例中，每位神经病理学家独立对16至19例进行两两分析，对5至6例进行组间分析。神经病理学家不知道研究设计，不熟悉分配给他们的病例，并且最初没有关于这些病例的临床信息。我们的结果表明，仅通过常规取样和染色方法，神经病理学检查不足以完全区分这些疾病。主要困难在于区分PSP的亚型以及将脑炎后帕金森综合征与PSP区分开来。皮质基底节变性和匹克病与PSP的区分相对较容易。添加最少的临床信息有助于提高诊断的准确性。基于所获得的结果，我们提出临床病理诊断标准以改进NINDS标准。
