Rapidly Progressive Dementia in the Outpatient Clinic: More Than Prions
Rapidly Progressive Dementia in the Outpatient Clinic: More Than Prions
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DOI：
10.1097/wad.0000000000000276
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发表时间：
2018-10-01
影响因子：
2.1
通讯作者：
Morris, John C.
中科院分区：
文献类型：
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作者：
Day, Gregory S.;Musiek, Erik S.;Morris, John C.
背景资料：已发表的评估和管理快速进展性痴呆（RPD）患者的方法在很大程度上借鉴了专门诊断克雅氏病的学术医院和国家中心的经验。这些方法是否可以应用于低敏度门诊患者的评估是未知的。研究方法：从2006年2月至2016年2月，在华盛顿大学医学院（圣刘易斯，MO）门诊记忆诊所评估了共计96例疑似RPD患者。由2名痴呆专家独立审查临床数据后，建立了一致的病因诊断。结果：总共有67/90（70%）例患者在症状发作后2年内表现出快于预期的认知下降，导致痴呆。女性（42/67，63%）、中位患者年龄（68.3岁;范围：45.4 - 89.6岁）和受教育年限（12岁;范围：6 - 14岁）与临床人口统计学一致。常见神经退行性痴呆的非典型表现占RPD病例的90%（60/67）。年龄越大，遗忘型阿尔茨海默病痴呆的几率越高（OR，2.1/10; 95%CI，1.1-3.8; P=0.02）。帕金森综合征（OR，6.9; 95% CI，1.6-30.5; P=0.01）或皮质视觉功能障碍（OR，10.8; 95% CI，1.7-69.4; P=0.01）预测RPD的另一种神经退行性原因（包括散发性克雅氏病）的几率较高。结论和相关性：临床环境影响RPD病因的患病率。临床评价应适应于促进RPD常见原因的检测，具体到实践环境。
Background: Published approaches to the evaluation and management of patients with rapidly progressive dementia (RPD) have been largely informed by experience at academic hospitals and national centers specializing in the diagnosis of Creutzfeldt-Jakob disease. Whether these approaches can be applied to patients assessed within lower-acuity outpatient settings is unknown. Methods: A total of 96 patients with suspected RPD were assessed within the Washington University School of Medicine (Saint Louis, MO) outpatient memory clinic from February 2006 to February 2016. Consensus etiologic diagnoses were established following independent review of clinical data by 2 dementia specialists. Results: In total, 67/90 (70%) patients manifested with faster-than-expected cognitive decline leading to dementia within 2 years of symptom onset. Female sex (42/67, 63%), median patient age (68.3 y; range, 45.4 to 89.6), and years of education (12 y; range, 6 to 14) were consistent with clinic demographics. Atypical presentations of common neurodegenerative dementing illnesses accounted for 90% (60/67) of RPD cases. Older age predicted a higher odds of amnestic Alzheimer disease dementia (OR, 2.1 per decade; 95% CI, 1.1-3.8; P=0.02). Parkinsonism (OR, 6.9; 95% CI, 1.6-30.5; P=0.01) or cortical visual dysfunction (OR, 10.8; 95% CI, 1.7-69.4; P=0.01) predicted higher odds of another neurodegenerative cause of RPD, including sporadic Creutzfeldt-Jakob disease. Conclusions and Relevance: The clinical environment influences the prevalence of RPD causes. The clinical evaluation should be adapted to promote detection of common causes of RPD, specific to the practice setting.