Pathophysiology and treatment of alien hand syndrome.
Pathophysiology and treatment of alien hand syndrome.
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DOI：
10.7916/d8vx0f48
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发表时间：
2014
期刊：
影响因子：
--
通讯作者：
Severt WL
中科院分区：
文献类型：
--
作者：
Sarva H;Deik A;Severt WL
异手综合征（AHS）是一种不自主但有目的的手部运动障碍，可能伴有失认症、失语、无力或感觉丧失。我们在此回顾大多数报告的病例，目前的病理生理和治疗的认识。我们在2014年7月进行了PubMed搜索，使用短语“异手综合征”，“异手综合征病理生理学”，“异手综合征治疗”和“无政府手综合征”。我们检索了141篇论文（综述、病例报告、病例系列和临床研究），其中我们回顾了109篇。没有英文摘要的非英文报告被排除在外。越来越多的证据表明，AHS有三种变体：额叶、胼胝体和后叶。患者可能表现出多种类型的症状；现象学和神经影像学之间缺乏相关性。大多数病理和功能成像研究表明，网络中断导致抑制丧失是可能的原因。成功的干预措施包括注射肉毒杆菌毒素、氯硝西泮、视觉空间指导技术、转移受影响的手的注意力和认知行为疗法。现有文献表明，AHS亚型之间的重叠是常见的。有效治疗的证据仍然是轶事，而且，鉴于AHS的罕见性，进行随机、安慰剂对照试验的可能性似乎不大。与许多其他运动障碍的干预措施一样，识别由AHS引起的特定功能障碍可能为个性化支持性护理提供最佳指导。
Alien hand syndrome (AHS) is a disorder of involuntary, yet purposeful, hand movements that may be accompanied by agnosia, aphasia, weakness, or sensory loss. We herein review the most reported cases, current understanding of the pathophysiology, and treatments. We performed a PubMed search in July of 2014 using the phrases “alien hand syndrome,” “alien hand syndrome pathophysiology,” “alien hand syndrome treatment,” and “anarchic hand syndrome.” The search yielded 141 papers (reviews, case reports, case series, and clinical studies), of which we reviewed 109. Non-English reports without English abstracts were excluded. Accumulating evidence indicates that there are three AHS variants: frontal, callosal, and posterior. Patients may demonstrate symptoms of multiple types; there is a lack of correlation between phenomenology and neuroimaging findings. Most pathologic and functional imaging studies suggest network disruption causing loss of inhibition as the likely cause. Successful interventions include botulinum toxin injections, clonazepam, visuospatial coaching techniques, distracting the affected hand, and cognitive behavioral therapy. The available literature suggests that overlap between AHS subtypes is common. The evidence for effective treatments remains anecdotal, and, given the rarity of AHS, the possibility of performing randomized, placebo-controlled trials seems unlikely. As with many other interventions for movement disorders, identifying the specific functional impairments caused by AHS may provide the best guidance towards individualized supportive care.