Clinical features and natural history of progressive supranuclear palsy - A clinical cohort study
Clinical features and natural history of progressive supranuclear palsy - A clinical cohort study
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DOI：
10.1212/01.wnl.0000052991.70149.68
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发表时间：
2003-03-25
期刊：
影响因子：
9.9
通讯作者：
Burn, DJ
中科院分区：
文献类型：
--
作者：
Nath, U;Ben-Shlomo, Y;Burn, DJ
目标：目的：探讨进行性核上性麻痹（PSP）的临床特点及预后因素。方法：对187例PSP患者进行记录式诊断。从患者记录中提取临床信息。研究者对62例患者（33%）进行了检查。62例患者中有49例（79%）接受了标准化临床评估。在平均6.4年后对生存预测因素进行了检查。结果：发病时最常见的症状与活动有关（69%）。在接受标准化临床评估的患者中，复视发生率为61%，眼恐怖发生率为43%，眼睑失用发生率为43%。随访期间死亡75例（40%）。发病年龄较大和分类为可能的PSP与较差的生存率相关。福尔斯发作（风险比3.28，95% CI 1.17 - 9.13），言语问题（风险比4.74，95% CI 1.10 - 20.4）或复视1年内出现吞咽困难（危险比4.23，95% CI 1.23 ~ 14.6）和2年内出现吞咽困难（危险比3.91，95% CI 1.39 ~ 11.0）与预后差相关。结论：活动性问题是PSP最常见的早期特征，视觉症状通常是功能性残疾。早期福尔斯、言语和吞咽问题、复视和早期插入经皮胃造口术可预测生存率降低。
Objective: To describe clinical features and identify prognostic predictors in progressive supranuclear palsy (PSP) Methods: Record-based diagnosis according to National Institute of Neurological Disorders and Stroke-Society for Progressive Supranuclear Palsy criteria was performed in 187 cases of PSP. Clinical information was abstracted from patient records. Sixty-two patients (33%) were examined by the investigators. Forty-nine of 62 patients (79%) underwent standardized clinical assessment. Predictors of survival were examined after a mean of 6.4 years. Results: The most common symptoms at disease onset related to mobility (69%). Of patients undergoing standardized clinical assessment, diplopia occurred in 61%, photophobia in 43%, and eyelid apraxia in 43%. Seventy-five cases (40%) died during follow-up. Older age at onset and classification as probable PSP were associated with poorer survival. Onset of falls (hazard ratio 3.28, 95% CI 1.17 to 9.13), speech problems (hazard ratio 4.74, 95% CI 1.10 to 20.4), or diplopia (hazard ratio 4.23, 95% CI 1.23 to 14.6) within 1 year and swallowing problems within 2 years (hazard ratio 3.91, 95% CI 1.39 to 11.0) were associated with a worse prognosis. Conclusions: Mobility problems are the commonest early feature in PSP and visual symptoms are often functionally disabling. Early falls, speech and swallowing problems, diplopia, and early insertion of a percutaneous gastrostomy predict reduced survival.