Progressive supranuclear palsy diagnosis and confounding features: Report on 16 autopsied cases
Progressive supranuclear palsy diagnosis and confounding features: Report on 16 autopsied cases
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DOI：
10.1002/mds.10211
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发表时间：
2002-11-01
影响因子：
8.6
通讯作者：
George, D
中科院分区：
文献类型：
--
作者：
Birdi, S;Rajput, AH;George, D
对1969-2000年31年间16例（男15例，女1例）经尸检证实的进行性核上性麻痹（PSP）的临床诊断和病程进行分析。首发症状为步态困难或姿势不稳定9例（56.3%），全身运动减慢3例（18.8%），震颤2例。1例患者发病时出现认知功能下降，1例患者出现偏侧妄想。4例首次评估时出现核上性眼肌麻痹（SNO），诊断为PSP。最后一次评估时，另外4例病例被诊断为PSP，但在8例（50%）从未表现出眼肌麻痹（平均发病后9.8年）的病例中，未被诊断为PSP。病程中有延髓症状13例（81.3%），认知功能障碍10例（62.5%）。15例病例接受左旋多巴、金刚烷胺、抗胆碱能药物、多巴胺激动剂和司来吉兰不同组合治疗，15例中有9例（60%）症状获益。5例患者在单独使用左旋多巴后有一定程度的改善，3例患者在左旋多巴中加入多巴胺激动剂后表现出更多的改善。总的来说，这种益处很小，并且只在疾病过程的早期发生。平均发病年龄为63.7岁（范围：5385岁）。PSP诊断的平均持续时间为4.8（范围，2-11）年。平均生存期为8.6（范围，3-24）年，平均死亡年龄为72.3（范围，60-89）岁。当使用文献中推荐的不同诊断标准时，临床诊断的准确性没有实质性提高。(C)2002运动障碍协会。
We evaluated 16 (15 men, I 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 I as hemidysionia. 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, 5385) 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. (C) 2002 Movement Disorder Society.