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路易体痴呆

[Dementia with Lewy bodies].

作者信息

Mori E

出版信息

Nihon Ronen Igakkai Zasshi. 2000 Oct;37(10):772-6. doi: 10.3143/geriatrics.37.772.

Abstract

Dementia with Lewy bodies (DLB), the second most frequent cause of primary degenerative dementias following Alzheimer's disease, has been increasingly recognized since the proposal of the consensus name and clinical diagnostic criteria. Although DLB overlaps in clinical, pathological, and genetic features with Alzheimer's disease and Parkinson's disease, DLB should be understood as an entity with the essential feature of the presence of Lewy bodies in the brain stem and cerebral cortex. From the clinical point of view, DLB is characterized by the presence of progressive dementia without severe memory disorders at the early stage, with significant cognitive fluctuations, well-formed recurrent visual hallucinations, and spontaneous Parkinsonism. This article reviews recent clinical and research findings, including our own, to facilitate clinical recognition of DLB. In addition to the supportive features described in the consortium clinical diagnostic criteria for DLB such as falls and great sensitivity to neuroleptic drugs, our studies found other frequent disorders including disproportionately severe visuoconstructive and visuoperceptual disturbances, transitory alterations in consciousness with reduplication phenomena, misidentification delusions, and non-aphasic misnamings. Neuroimaging features include relatively preserved hippocampal volume on MRI and occipital involvement on metabolic and blood flow imagings. The correct diagnosis of DLB is important to administer adequate treatment, to avoid adverse effects with neuroleptic drugs, and to establish precise prognosis. The present summary of the clinical features is hopefully helpful for clinical diagnosis of DLB. From a therapeutic point of view, cholinesterase inhibitors seemingly show some efficacy in the treatment of cognitive alterations. Further research would result in advances in diagnostic methods and therapeutic approaches in the near future.

摘要

路易体痴呆(DLB)是继阿尔茨海默病之后第二常见的原发性退行性痴呆病因,自其共识名称和临床诊断标准提出以来,已得到越来越多的认可。尽管DLB在临床、病理和遗传特征上与阿尔茨海默病和帕金森病存在重叠,但应将其理解为一种以脑干和大脑皮质存在路易小体为基本特征的疾病实体。从临床角度来看,DLB的特点是早期出现进行性痴呆且无严重记忆障碍,伴有明显的认知波动、逼真的反复视幻觉和自发性帕金森综合征。本文回顾了包括我们自己的研究在内的近期临床和研究发现,以促进对DLB的临床识别。除了DLB联盟临床诊断标准中描述的支持性特征,如跌倒和对神经安定药物高度敏感外,我们的研究还发现了其他常见病症,包括不成比例的严重视觉构建和视觉感知障碍、伴有复制现象的意识短暂改变、错认妄想和非失语性错名。神经影像学特征包括MRI上相对保留的海马体积以及代谢和血流成像上的枕叶受累。正确诊断DLB对于给予适当治疗、避免神经安定药物的不良反应以及确定准确预后非常重要。目前对临床特征的总结有望有助于DLB的临床诊断。从治疗角度来看,胆碱酯酶抑制剂似乎在治疗认知改变方面显示出一定疗效。进一步的研究将在不久的将来推动诊断方法和治疗方法的进步。

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