The clinical characteristics of dementia with Lewy bodies and a consideration of prodromal diagnosis.

The clinical characteristics of dementia with Lewy bodies and a consideration of prodromal diagnosis.
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DOI:
10.1186/alzrt274
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发表时间:
2014
期刊:
Alzheimer's research & therapy
影响因子:
--
通讯作者:
McKeith IG
McKeith IG
中科院分区:
其他
文献类型:
--
作者:
Donaghy PC;McKeith IG

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路易体痴呆(DLB)是继阿尔茨海默病(AD)之后的第二种最常见的退行性痴呆。DLB在临床和病理上与帕金森病(PD)和PD痴呆相关,这三种疾病可以被视为存在于路易体病的一个谱中。近年来,人们一直在努力确定AD和PD在前驱期的表型(在表达认知和运动障碍的相应综合征之前)。DLB的前驱表现的证据也正在出现。本文简要回顾了什么是已知的前驱DLB的临床表现之前,讨论路易体病的病理,这是如何与前驱DLB的潜在生物标志物。DLB的表现特征可以大致分为三类:认知障碍(特别是非遗忘性认知障碍)、行为/精神现象(例如幻觉、快速眼动睡眠行为障碍(RBD))和身体症状(例如帕金森综合征、嗅觉下降、自主神经功能障碍)。一些非认知症状,如便秘,RBD,嗅觉减退和体位性头晕,可以提前数年的记忆障碍的发作在DLB。路易体病的病理学研究发现,最早受累的部位是嗅球、迷走神经背侧运动核、周围自主神经系统(包括肠神经系统)和脑干。DLB的一些最有希望的早期标志物包括RBD的存在、自主神经功能障碍或嗅觉减退、123 I-间碘苄胍心脏血管造影、黑质病理学测量和外周自主神经中α-突触核蛋白的皮肤活检。在缺乏疾病修饰疗法的情况下,前驱DLB的诊断在临床上的用途有限。也就是说,DLB前驱发展的知识可以帮助临床医生识别诊断不确定的DLB病例。前驱诊断在研究中非常重要,在早期阶段识别路易体病可以使研究人员研究痴呆病理生理学的初始阶段,开发旨在中断痴呆综合征发展的治疗方法,并准确识别最有可能从这些治疗中受益的患者。
Dementia with Lewy bodies (DLB) is the second most common type of degenerative dementia following Alzheimer’s disease (AD). DLB is clinically and pathologically related to Parkinson's disease (PD) and PD dementia, and the three disorders can be viewed as existing on a spectrum of Lewy body disease. In recent years there has been a concerted effort to establish the phenotypes of AD and PD in the prodromal phase (before the respective syndromes of cognitive and motor impairment are expressed). Evidence for the prodromal presentation of DLB is also emerging. This paper briefly reviews what is known about the clinical presentation of prodromal DLB before discussing the pathology of Lewy body disease and how this relates to potential biomarkers of prodromal DLB. The presenting features of DLB can be broadly placed in three categories: cognitive impairment (particularly nonamnestic cognitive impairments), behavioural/psychiatric phenomena (for example, hallucinations, rapid eye movement sleep behaviour disorder (RBD)) and physical symptoms (for example, parkinsonism, decreased sense of smell, autonomic dysfunction). Some noncognitive symptoms such as constipation, RBD, hyposmia and postural dizziness can predate the onset of memory impairment by several years in DLB. Pathological studies of Lewy body disease have found that the earliest sites of involvement are the olfactory bulb, the dorsal motor nucleus of the vagal nerve, the peripheral autonomic nervous system, including the enteric nervous system, and the brainstem. Some of the most promising early markers for DLB include the presence of RBD, autonomic dysfunction or hyposmia, 123I-metaiodobenzylguanidine cardiac scintigraphy, measures of substantia nigra pathology and skin biopsy for α-synuclein in peripheral autonomic nerves. In the absence of disease-modifying therapies, the diagnosis of prodromal DLB is of limited use in the clinic. That said, knowledge of the prodromal development of DLB could help clinicians identify cases of DLB where the diagnosis is uncertain. Prodromal diagnosis is of great importance in research, where identifying Lewy body disease at an earlier stage may allow researchers to investigate the initial phases of dementia pathophysiology, develop treatments designed to interrupt the development of the dementia syndrome and accurately identify the patients most likely to benefit from these treatments.
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发表时间: 2011-05
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影响因子: --
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