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路易体痴呆

Lewy body dementia.

作者信息

Hanson Jennifer C, Lippa Carol F

机构信息

Drexel University College of Medicine, Mail Stop 423, Philadelphia, Pennsylvania 19107, USA.

出版信息

Int Rev Neurobiol. 2009;84:215-28. doi: 10.1016/S0074-7742(09)00411-5.

Abstract

Dementia is becoming increasingly prevalent since elderly patients are living longer due to the development of treatments for other diseases and conditions. The percent of our population over 60 is also increasing with the wave of aging baby boomers. Additionally, more individuals seek medical assistance for cognitive problems as visibility for treatments improves. This combination of factors results in the dementia syndromes becoming more common, causing physicians to encounter more patients with dementia as well as more caregivers of these patients. Of dementia subtypes, Alzheimer's disease (AD) is the most common. Dementia with Lewy bodies (DLB) is thought to be the second most common subtype. DLB's typical symptoms include cognitive impairment, visual hallucinations, spontaneous parkinsonism, and fluctuating confusion. Supportive features include a variety of sleep disruptions that may occur before manifestations of dementia. Psychiatric symptoms include vivid visual hallucinations and depression. The clinical features of DLB are strikingly similar to those of dementia in Parkinson's disease (PD). The underlying biology of DLB is complex, but the presence of alpha-synuclein containing Lewy bodies (LB) is a common factor. These inclusions also contain ubiquitin. PD dementia shares these pathological findings with DLB, as well as neural degeneration of the substantia nigra. DLB and dementia in PD may represent the same pathological process along a disease spectrum. Additionally, many DLB cases are also associated with beta-amyloid and tau-containing neurofibrillary tangles, features that are associated with AD. Frequently, AD patients are also found to have LB. The reason for this overlap is unknown. However, the greater the Alzheimer's pathology in DLB patients, the more the clinical features of DLB overlaps with AD. In this chapter, we will review DLB including clinical, pathological, and radiological features as well as biomarkers and treatments.

摘要

由于其他疾病和病症的治疗取得进展,老年患者的寿命延长,痴呆症正变得越来越普遍。随着婴儿潮一代的老龄化浪潮,我国60岁以上人口的比例也在增加。此外,随着治疗的知名度提高,越来越多的人因认知问题寻求医疗帮助。这些因素共同导致痴呆综合征变得更加常见,使得医生遇到更多的痴呆患者以及这些患者的更多护理人员。在痴呆亚型中,阿尔茨海默病(AD)最为常见。路易体痴呆(DLB)被认为是第二常见的亚型。DLB的典型症状包括认知障碍、视幻觉、自发性帕金森症和波动性意识模糊。支持性特征包括在痴呆症状出现之前可能发生的各种睡眠障碍。精神症状包括生动的视幻觉和抑郁。DLB的临床特征与帕金森病(PD)中的痴呆症极为相似。DLB的潜在生物学机制很复杂,但含有α-突触核蛋白的路易小体(LB)的存在是一个共同因素。这些包涵体还含有泛素。PD痴呆与DLB有这些共同的病理发现,以及黑质的神经变性。DLB和PD中的痴呆可能代表疾病谱上相同的病理过程。此外,许多DLB病例还与β-淀粉样蛋白和含tau的神经原纤维缠结有关,这些特征与AD相关。通常,AD患者也被发现有LB。这种重叠的原因尚不清楚。然而,DLB患者中阿尔茨海默病病理特征越明显,DLB的临床特征与AD的重叠就越多。在本章中,我们将回顾DLB,包括临床、病理、放射学特征以及生物标志物和治疗方法。

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