Alien limb in the corticobasal syndrome: phenomenological characteristics and relationship to apraxia

Alien limb in the corticobasal syndrome: phenomenological characteristics and relationship to apraxia
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DOI:
10.1007/s00415-019-09672-8
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发表时间:
2020-04-01
影响因子:
6
通讯作者:
Rowe, James B.
Rowe, James B.
中科院分区:
医学2区
文献类型:
--
作者:
Lewis-Smith, David J.;Wolpe, Noham;Rowe, James B.

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异肢指的是看似有目的的动作,但与患者报告的意图无关。在皮质基底综合征中,异肢常与失用症同时发生,解剖学和现象学的比较表明,异肢和失用症可能与目标导向运动的失败有因果关系。在这里,我们描述了皮质基底动脉综合征患者(n = 30)的异肢症状的性质及其与肢体失用症的关系。25例进行性核上性麻痹Richardson综合征患者作为疾病对照组。结构化检查的实践,运动功能,认知和外国肢体参加区域专家诊所的患者进行。28例皮质基底动脉综合征患者(93%)表现出明显的失用症,这通常是不对称的,23/30例(77%)患者的左手优先受影响。此外,25/30例(83%)患者报告了一种或多种与异肢一致的症状。这些现象的范围很广,包括所有权和控制感的变化以及不受欢迎的流动。回归分析显示,肢体失用症的严重程度与异肢的发生或异肢现象的报道数量之间没有显着关联。贝叶斯估计表明,异肢和失用症之间的正相关概率较低,这表明异肢现象不太可能与严重失用症有关。我们的研究结果揭示了这些致残和尚未治愈的临床特征的现象学,与自愿行动的理论模型相关。
Alien limb refers to movements that seem purposeful but are independent of patients' reported intentions. Alien limb often co-occurs with apraxia in the corticobasal syndrome, and anatomical and phenomenological comparisons have led to the suggestion that alien limb and apraxia may be causally related as failures of goal-directed movements. Here, we characterised the nature of alien limb symptoms in patients with the corticobasal syndrome (n = 30) and their relationship to limb apraxia. Twenty-five patients with progressive supranuclear palsy Richardson syndrome served as a disease control group. Structured examinations of praxis, motor function, cognition and alien limb were undertaken in patients attending a regional specialist clinic. Twenty-eight patients with corticobasal syndrome (93%) demonstrated significant apraxia and this was often asymmetrical, with the left hand preferentially affected in 23/30 (77%) patients. Moreover, 25/30 (83%) patients reported one or more symptoms consistent with alien limb. The range of these phenomena was broad, including changes in the sense of ownership and control as well as unwanted movements. Regression analyses showed no significant association between the severity of limb apraxia and either the occurrence of an alien limb or the number of alien limb phenomena reported. Bayesian estimation showed a low probability for a positive association between alien limb and apraxia, suggesting that alien limb phenomena are not likely to be related to severity apraxia. Our results shed light on the phenomenology of these disabling and as yet untreatable clinical features, with relevance to theoretical models of voluntary action.