De Bruin V M, Lees A J
National Hospital for Neurology and Neurosurgery, London, England.
Mov Disord. 1994 Jul;9(4):381-9. doi: 10.1002/mds.870090402.
We have reviewed 90 patients from the literature with histopathological features compatible with currently accepted criteria for the diagnosis of the Steele-Richardson-Olszewski syndrome (SROS). Only 62 patients (69%) had clinically definite SROS based on the criteria of Maher and Lees. Neurofibrillary degeneration of subcortical structures with involvement of the internal pallidum, the subthalamic nucleus, and substantia nigra was considered essential for the pathological diagnosis. Thirty-six cases (40%) had neocortical neurofibrillary change that bore no clear relationship to the degree of cognitive impairment (chi 2 = 9.293; p < 0.4107). Grumose degeneration of the dentate nucleus was present in 25 cases (28%), and occasionally there were other, less usual, findings such as Pick bodies and Lewy bodies.
我们回顾了文献中90例组织病理学特征符合目前公认的斯蒂尔-理查森-奥尔谢夫斯基综合征（SROS）诊断标准的患者。根据马赫和李斯的标准，只有62例患者（69%）临床上确诊为SROS。病理诊断认为，伴有苍白球内侧部、丘脑底核和黑质受累的皮质下结构神经原纤维变性至关重要。36例（40%）有新皮质神经原纤维改变，这与认知障碍程度无明确关系（卡方检验=9.293；p<0.4107）。25例（28%）存在齿状核颗粒样变性，偶尔还有其他不太常见的表现，如皮克小体和路易小体。
