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.
- movement disorders
- tourette syndrome
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Twitter @YuliaWorbe, @ChristosGanos
Correction notice This article has been corrected since it appeared Online First. Minor text changes have been made, and Figure 2 replaced.
Contributors Study design: CG, J-FF, TM and TP. Study supervision: CG. Study selection: J-FF, TM and TP. Data extraction: J-FF and TM. Initial draft: CG and J-FF. Revision of the draft for important intellectual content: J-FF, TM, YW, TP, KB and CG. Interpretation of results: J-FF, TM, YW, TP, KB and CG.
Funding This study was funded by Volkswagen Foundation.
Competing interests TM was supported by the BIH-Charité Clinician Scientist Program of the Charité-Universitätsmedizin Berlin and the Berlin Institute of Health. YW was supported by an Agence National de Recherche grant, Dystonia Medical Research Foundation and a travel grant from Merz. KB received grant support form EU 2020 horizon, National Institute of Health Research/Research for Patient Benefit, Wellcome/MRC and PD UK, and received honoraria/financial support to speak/attend meetings from Ipsen, Merz, Sun Pharma, Allergan, Teva Lundbeck and UCB. KB received royalties from Oxford University Press and a stipend for Movement Disorders Clinical Practice editorship. CG received research grants from the VolkswagenStiftung (Freigeist Fellowship) and the German Parkinson Society and was also supported by the Deutsche Forschungsgemeinschaft (GA2031/1-1 and GA2031/1-2).
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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