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特发性 REM 睡眠行为障碍与神经退行性风险:告知还是不告知患者?如何将风险最小化?

Idiopathic REM sleep behavior disorder and neurodegenerative risk: To tell or not to tell to the patient? How to minimize the risk?

机构信息

Clinical Neurology, Department of Neuroscience (DINOGMI), University of Genoa, Italy.

Department of Biomedical and Neuromotor Sciences, Alma Mater Studiorum, University of Bologna, Italy.

出版信息

Sleep Med Rev. 2017 Dec;36:82-95. doi: 10.1016/j.smrv.2016.11.002. Epub 2016 Nov 10.

Abstract

Most people with idiopathic REM sleep behavior disorder (iRBD) have an underlying synucleinopathy, mainly Parkinson's disease (PD) or dementia with Lewy bodies, with median conversion time of 4-9 y from iRBD diagnosis and of 11-16 y from symptom onset. Subtle signs and imaging tests indicate concomitant neurodegeneration in widespread brain areas. Risk factor studies suggest that iRBD patients may have prior head injury, occupational farming, pesticide exposure, low education level and possibly more frequent family history of dream-enactment behavior (but not of PD), plus unexpected risk factors (smoking, ischemic heart disease and inhaled corticosteroid use). Unlike PD, caffeine and smoking appear not to have a protective role. Prior depression and antidepressant use may be early neurodegenerative signs rather than exclusively causative factors. Age, hyposmia, impaired color vision, abnormal dopaminergic imaging, mild cognitive impairment and possibly sleepiness, may identify patients at greater risk of more rapid conversion. The consensus is to generally disclose the neurodegenerative risk to patients (with the caveat that phenoconversion and its temporal course remain uncertain in individuals without "soft neurodegenerative signs" and those under 50 y of age), to suggest a healthy lifestyle and to take part in prospective cohort studies in anticipation of eventual neuroprotective trials.

摘要

大多数特发性 REM 睡眠行为障碍(iRBD)患者存在潜在的突触核蛋白病,主要为帕金森病(PD)或路易体痴呆,从中枢 REM 睡眠行为障碍(iRBD)诊断到出现症状的平均转化时间为 4-9 年,从症状出现到出现症状的平均转化时间为 11-16 年。细微的体征和影像学检查表明,广泛的大脑区域存在并发的神经退行性变。危险因素研究表明,iRBD 患者可能存在既往头部外伤、职业性农业劳动、农药暴露、低教育程度,并且可能有更频繁的梦境行为家族史(但不是 PD),加上一些意想不到的危险因素(吸烟、缺血性心脏病和吸入皮质类固醇的使用)。与 PD 不同,咖啡因和吸烟似乎没有保护作用。既往抑郁和抗抑郁药的使用可能是早期神经退行性的迹象,而不仅仅是唯一的致病因素。年龄、嗅觉减退、色觉异常、多巴胺能成像异常、轻度认知障碍,可能还有嗜睡,可能会识别出有更高风险、更快转化的患者。目前的共识是,一般向患者披露神经退行性风险(需要注意的是,在没有“软性神经退行性体征”且年龄在 50 岁以下的个体中,表型转化及其时间过程仍然不确定),建议健康的生活方式,并参与前瞻性队列研究,以期最终进行神经保护试验。

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