Davis P H, Bergeron C, McLachlan D R
Ann Neurol. 1985 Apr;17(4):337-43. doi: 10.1002/ana.410170406.
Four pathologically documented cases of progressive supranuclear palsy are reported. Two patients exhibited severe dementia and 2 parkinsonism; none had the classic ophthalmoplegia. On retrospective analysis, clues to the diagnosis included early prominent gait disturbance, apraxia of eyelid opening in 1 patient, lack of tremor, poor response to levodopa-carbidopa, and severe rigidity with a posture of neck extension terminally in 1 patient. The clinical presentation of progressive supranuclear palsy, therefore, is not as stereotyped as previously thought, and the diagnosis can be overlooked if one adheres rigidly to the classic diagnostic criteria.
报告了4例经病理证实的进行性核上性麻痹病例。2例患者表现为严重痴呆，2例表现为帕金森症；均无典型的眼肌麻痹。回顾性分析发现，诊断线索包括早期明显的步态障碍、1例患者存在眼睑开合失用、无震颤、对左旋多巴-卡比多巴反应不佳以及1例患者终末期出现严重强直并伴有颈部伸展姿势。因此，进行性核上性麻痹的临床表现并不像之前认为的那样具有刻板性，如果严格遵循经典诊断标准，可能会漏诊。
