Department of Neurology, Charité University Hospital Berlin, Berlin, Germany.
Berlin Institute of Health, Berlin, Germany.
J Neurol Neurosurg Psychiatry. 2020 Jul;91(7):712-719. doi: 10.1136/jnnp-2019-322569. Epub 2020 May 19.
Self-injurious behaviours (SIBs) are defined as deliberate, repetitive and persistent behaviours that are directed towards the body and lead to physical injury and are not associated with sexual arousal and without suicidal intent. In movement disorders, SIBs are typically associated with tic disorders, most commonly Tourette syndrome, and neurometabolic conditions, such as classic Lesch-Nyhan syndrome. However, beyond these well-known aetiologies, a range of other movement disorder syndromes may also present with SIBs, even though this clinical association remains less well-known. Given the scarcity of comprehensive works on this topic, here we performed a systematic review of the literature to delineate the spectrum of movement disorder aetiologies associated with SIBs. We report distinct aetiologies, which are clustered in five different categorical domains, namely, neurodevelopmental, neurometabolic and neurodegenerative disorders, as well as disorders with characteristic structural brain changes and heterogeneous aetiologies (eg, autoimmune and drug-induced). We also provide insights in the pathophysiology of SIBs in these patients and discuss neurobiological key risk factors, which may facilitate their manifestation. Finally, we provide a list of treatments, including practical measures, such as protective devices, as well as behavioural interventions and pharmacological and neurosurgical therapies.
自伤行为(SIBs)被定义为蓄意、重复和持续的行为,这些行为针对身体并导致身体伤害,与性唤起无关,也没有自杀意图。在运动障碍中,SIBs 通常与抽动障碍有关,最常见的是妥瑞氏综合征,以及神经代谢疾病,如经典的莱施-尼汉综合征。然而,除了这些众所周知的病因之外,一系列其他运动障碍综合征也可能伴有 SIBs,尽管这种临床关联不太为人所知。鉴于关于这个主题的综合作品很少,我们在这里对文献进行了系统回顾,以描绘与 SIBs 相关的运动障碍病因谱。我们报告了不同的病因,这些病因聚类在五个不同的类别域中,即神经发育、神经代谢和神经退行性疾病,以及具有特征性结构脑改变和异质性病因的疾病(例如自身免疫和药物诱导的疾病)。我们还提供了这些患者 SIBs 的发病机制的见解,并讨论了神经生物学的关键风险因素,这些因素可能促进其表现。最后,我们提供了一系列治疗方法,包括实用措施,如防护装置,以及行为干预和药物治疗和神经外科治疗。