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[阿尔茨海默病的临床异质性。不同的临床特征可预测进展速度]

[Clinical heterogeneity of Alzheimer's disease. Different clinical profiles can predict the progression rate].

作者信息

Mangone C A

机构信息

Centro de Asistencia e Investigación del Deterioro Cognitivo, Hospital Santojani, Buenos Aires, Argentina.

出版信息

Rev Neurol. 2004;38(7):675-81.

Abstract

INTRODUCTION

Alzheimer's disease (AD) is a degenerative dementia that may disclose different cognitive, behavioral, psychiatric and functional symptoms since onset. These distinct cognitive profiles support the conception of clinical heterogeneity and account for AD's highly variable rate of progression. In spite of strict diagnostic criteria NINCS ADRDA's and DSM IV the clinical certainty is only about 85%. Mayeux define 4 subtypes: a). Benign: mild cognitive and functional impairment without focal signs and late onset behavioral signs, slow progression; b). Myoclonic: usually of presenile onset with severe cognitive deterioration, mutism and early onset myoclonus; c). Extrapyramidal: early onset akineto rigid signs with severe cognitive, behavioral and psychiatric involvement; d). Typical: gradual and progressive cognitive, behavioral and functional impairment. The differentiation of these subtypes will allow us to define discrete patterns of progression, to define prognostic subgroups, and to homogenize them for clinical research and drug trials.

DEVELOPMENT

We examined 1000 charts of probable AD patients from the Santojanni Center. We found 42% extrapyramidal, 35% typical, 15% benign and 8% myoclonic. The early onset of parkinsonism and myoclonus predict a rapidly evolving cognitive impairment and a more severe rate of progression with psychiatric disorders and dependency in activities of daily living. (DADL) Patients with low level of education, low cognitive performance at entry as well as those with rapid rate of cognitive deterioration had a faster rate of progression to DADL.

CONCLUSION

Delusions, low level of education, extrapyramidal signs and motor hyperactivity but not hallucinations, and anosognosia were the best non cognitive predictors of DADL.

摘要

引言

阿尔茨海默病(AD)是一种退行性痴呆,自发病起可能会出现不同的认知、行为、精神和功能症状。这些不同的认知特征支持了临床异质性的概念,并解释了AD高度可变的进展速度。尽管有严格的诊断标准(NINCS ADRDA和DSM IV),临床确诊率仅约为85%。Mayeux定义了4种亚型:a)良性:轻度认知和功能障碍,无局灶性体征和迟发性行为体征,进展缓慢;b)肌阵挛性:通常为早发性,伴有严重认知恶化、缄默和早发性肌阵挛;c)锥体外系性:早发性运动不能-强直体征,伴有严重认知、行为和精神方面受累;d)典型性:逐渐进展的认知、行为和功能障碍。区分这些亚型将使我们能够定义不同的进展模式,确定预后亚组,并使其在临床研究和药物试验中同质化。

发展

我们检查了圣托亚尼中心1000例可能患有AD患者的病历。我们发现42%为锥体外系性,35%为典型性,15%为良性,8%为肌阵挛性。帕金森病和肌阵挛的早发预示着认知障碍迅速发展,以及在精神障碍和日常生活活动依赖方面更严重的进展速度。(DADL)教育水平低、入院时认知表现差以及认知恶化速度快的患者向DADL进展的速度更快。

结论

妄想、低教育水平、锥体外系体征和多动但不是幻觉以及疾病感缺失是DADL的最佳非认知预测因素。

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