Birdi Sam, Rajput Ali H, Fenton Mark, Donat Jeffery R, Rozdilsky Bohdan, Robinson Christopher, Macaulay Rob, George David
Division of Neurology, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.
Mov Disord. 2002 Nov;17(6):1255-64. doi: 10.1002/mds.10211.
We evaluated 16 (15 men, 1 woman) autopsy-verified progressive supranuclear palsy (PSP) cases during 31 years (1969-2000) for clinical diagnosis and the course of the disease. The onset was gait difficulty or postural instability in 9 (56.3%), general motor slowing in 3 (18.8%), and tremor in 2. One case had onset with cognitive decline and 1 as hemidystonia. Four cases had supranuclear ophthalmoplegia (SNO) at the first assessment and were diagnosed as PSP. By last assessment, PSP diagnosis was made in 4 additional cases, but in 8 (50%) who never manifested ophthalmoplegia (mean 9.8 years after onset), PSP diagnosis was not made. Other manifestations included bulbar symptoms in 13 (81.3%), and cognitive impairment in 10 (62.5%) during the course of illness. Fifteen cases received levodopa, amantadine, anticholinergics, dopamine agonists, and selegiline in different combinations with symptomatic benefit in 9 of 15 (60%). Five had some improvement on levodopa alone and 3 showed more improvement when a dopamine agonist was added to levodopa. In general, the benefit was minimal and occurred only early in the course of illness. The mean age at onset was 63.7 (range, 53-85) years. Mean duration at PSP diagnosis was 4.8 (range, 2-11) years. Mean survival was 8.6 (range, 3-24) years and mean age at death was 72.3 (range, 60-89) years. When the different diagnostic criteria recommended in the literature were used, the accuracy of clinical diagnosis did not improve substantially.
在31年（1969 - 2000年）间，我们对16例（15名男性，1名女性）经尸检证实的进行性核上性麻痹（PSP）病例进行了临床诊断及疾病病程评估。9例（56.3%）起病表现为步态困难或姿势不稳，3例（18.8%）为全身运动迟缓，2例为震颤。1例以认知功能减退起病，1例以偏身肌张力障碍起病。4例在首次评估时出现核上性眼肌麻痹（SNO）并被诊断为PSP。至末次评估时，又有4例被诊断为PSP，但8例（50%）从未出现眼肌麻痹（起病后平均9.8年）者未被诊断为PSP。其他表现包括病程中13例（81.3%）出现球部症状，10例（62.5%）出现认知障碍。15例患者接受了左旋多巴、金刚烷胺、抗胆碱能药物、多巴胺激动剂和司来吉兰的不同联合治疗，15例中有9例（60%）有症状改善。5例仅使用左旋多巴有一定改善，3例在左旋多巴基础上加用多巴胺激动剂后改善更明显。总体而言，获益极小且仅在病程早期出现。起病时的平均年龄为63.7岁（范围53 - 85岁）。PSP诊断时的平均病程为4.8年（范围2 - 11年）。平均生存期为8.6年（范围3 - 24年），死亡时的平均年龄为72.3岁（范围60 - 89岁）。当采用文献中推荐的不同诊断标准时，临床诊断的准确性并未显著提高。
