The improvement of movement and speech during rapid eye movement sleep behaviour disorder in multiple system atrophy
The improvement of movement and speech during rapid eye movement sleep behaviour disorder in multiple system atrophy
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DOI：
10.1093/brain/awq379
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发表时间：
2011-03-01
期刊：
影响因子：
14.5
通讯作者：
Arnulf, Isabelle
中科院分区：
文献类型：
--
作者：
De Cock, Valerie Cochen;Debs, Rachel;Arnulf, Isabelle
多系统萎缩是一种以左旋多巴反应不良的严重运动障碍为特征的非典型帕金森病。大多数患者出现快速眼动睡眠行为障碍。由于帕金森病患者在快速眼动睡眠行为障碍中不存在帕金森症状，我们研究了多系统萎缩患者在快速眼动睡眠中的运动。采用结构化问卷对49名非痴呆多系统萎缩患者和49名特发性帕金森病患者及其98名床伴进行了访谈。他们将快速眼动睡眠行为障碍期间的运动、声音和面部表情的质量评为比清醒状态下的相同活动好、等于或更差。对22/49多系统萎缩患者和19/49帕金森病患者的睡眠和运动进行视频多导睡眠描记术监测。这些记录分析了在快速眼动睡眠过程中帕金森症和小脑综合征的存在。43/49例（88%）多系统萎缩患者存在临床快速眼动睡眠行为障碍。来自31/43位能够评估睡眠中运动的床伴的报告表明，81%的患者在快速眼动睡眠行为障碍中表现出某种形式的改善。其中包括改善运动（73%的患者：更快，67%；更强壮，52%；更流畅，26%），改善语言（59%的患者：更大声，55%；更清晰，17%；更清晰，36%）和正常化的面部表情（50%的患者）。帕金森病的改善率高于多系统萎缩，但两种形式的多系统萎缩（主要帕金森病与小脑综合征）之间没有进一步的差异。多系统萎缩患者在快速眼动睡眠期间的运动视频监测显示，与清醒时的面部表情和运动相比，他们的面部表情更富有表情，运动速度更快，更丰富。这些动作仍然有些不稳定，但没有明显的帕金森症状。小脑体征无法评估。我们得出结论，帕金森病在多系统萎缩患者的快速眼动睡眠行为障碍中也会消失，但这种改善不是由于多巴胺传递增强，因为这些患者对左旋多巴不敏感。这些数据表明，这些运动不受锥体外区影响；然而，小脑控制异常的影响尚不清楚。帕金森氏症的短暂消失更令人惊讶，因为没有任何治疗（甚至是多巴胺能）对这种致残疾病提供真正的好处。
Multiple system atrophy is an atypical parkinsonism characterized by severe motor disabilities that are poorly levodopa responsive. Most patients develop rapid eye movement sleep behaviour disorder. Because parkinsonism is absent during rapid eye movement sleep behaviour disorder in patients with Parkinson's disease, we studied the movements of patients with multiple system atrophy during rapid eye movement sleep. Forty-nine non-demented patients with multiple system atrophy and 49 patients with idiopathic Parkinson's disease were interviewed along with their 98 bed partners using a structured questionnaire. They rated the quality of movements, vocal and facial expressions during rapid eye movement sleep behaviour disorder as better than, equal to or worse than the same activities in an awake state. Sleep and movements were monitored using video-polysomnography in 22/49 patients with multiple system atrophy and in 19/49 patients with Parkinson's disease. These recordings were analysed for the presence of parkinsonism and cerebellar syndrome during rapid eye movement sleep movements. Clinical rapid eye movement sleep behaviour disorder was observed in 43/49 (88%) patients with multiple system atrophy. Reports from the 31/43 bed partners who were able to evaluate movements during sleep indicate that 81% of the patients showed some form of improvement during rapid eye movement sleep behaviour disorder. These included improved movement (73% of patients: faster, 67%; stronger, 52%; and smoother, 26%), improved speech (59% of patients: louder, 55%; more intelligible, 17%; and better articulated, 36%) and normalized facial expression (50% of patients). The rate of improvement was higher in Parkinson's disease than in multiple system atrophy, but no further difference was observed between the two forms of multiple system atrophy (predominant parkinsonism versus cerebellar syndrome). Video-monitored movements during rapid eye movement sleep in patients with multiple system atrophy revealed more expressive faces, and movements that were faster and more ample in comparison with facial expression and movements during wakefulness. These movements were still somewhat jerky but lacked any visible parkinsonism. Cerebellar signs were not assessable. We conclude that parkinsonism also disappears during rapid eye movement sleep behaviour disorder in patients with multiple system atrophy, but this improvement is not due to enhanced dopamine transmission because these patients are not levodopa-sensitive. These data suggest that these movements are not influenced by extrapyramidal regions; however, the influence of abnormal cerebellar control remains unclear. The transient disappearance of parkinsonism here is all the more surprising since no treatment (even dopaminergic) provides a real benefit in this disabling disease.