Osaki Yasushi, Ben-Shlomo Yoav, Lees Andrew J, Daniel Susan E, Colosimo Carlo, Wenning Gregor, Quinn Niall
National Hospital for Neurology and Neurosurgery, London, United Kingdom.
Mov Disord. 2004 Feb;19(2):181-9. doi: 10.1002/mds.10680.
We assessed the accuracy of clinical diagnosis of progressive supranuclear palsy (PSP, Steele-Richardson-Olszewski disease) and the validity of existing sets of clinical diagnostic criteria for PSP (see Appendix) using neuropathologically examined cases from the Queen Square Brain Bank for Neurological Disorders. Diagnosis of PSP was made by 40 different physicians, and 60 cases clinically diagnosed as PSP when last assessed in life were studied. In 47 cases (78%), the diagnosis of PSP was confirmed pathologically. False-positive diagnoses included Parkinson's disease with significant additional cortical Lewy body (n = 3) or Alzheimer (n = 1) pathology, multiple system atrophy (n = 4), and corticobasal degeneration, Pick's disease, motor neurone disease, cerebrovascular disease, and a sporadic case of frontotemporal dementia and parkinsonism linked to chromosome 17 (1 case each). Most cases of PSP were diagnosed accurately by neurologists at the final assessment. Although application of National Institute of Neurological Disorders and the Society for PSP possible category marginally improved the accuracy of initial clinical diagnosis, none of the existing operational criteria could significantly improve accuracy of the final clinical diagnosis.
我们利用来自女王广场神经疾病脑库经神经病理学检查的病例，评估了进行性核上性麻痹（PSP，即斯蒂尔-理查森-奥尔谢夫斯基病）临床诊断的准确性以及现有PSP临床诊断标准集（见附录）的有效性。40位不同的医生对PSP进行了诊断，并对60例生前最后一次评估时临床诊断为PSP的病例进行了研究。在47例（78%）病例中，PSP的诊断经病理证实。假阳性诊断包括伴有显著额外皮质路易体的帕金森病（n = 3）或阿尔茨海默病（n = 1）病理、多系统萎缩（n = 4）、皮质基底节变性、皮克病、运动神经元病、脑血管病以及1例与17号染色体相关的散发性额颞叶痴呆和帕金森综合征病例。大多数PSP病例在最终评估时由神经科医生准确诊断。尽管应用美国国立神经疾病研究所和PSP协会的可能分类在一定程度上提高了初始临床诊断的准确性，但现有的任何操作标准都不能显著提高最终临床诊断的准确性。
