• Open Access

Limb amputations in fixed dystonia: A form of body integrity identity disorder?

Authors

  • Mark J. Edwards PhD,

    1. Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, UCL, London, United Kingdom
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    • Mark J. Edwards and Araceli Alonso-Canovas contributed equally to this work.

  • Araceli Alonso-Canovas MD,

    1. Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, UCL, London, United Kingdom
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    • Mark J. Edwards and Araceli Alonso-Canovas contributed equally to this work.

  • Arnette Schrag PhD,

    1. Department of Clinical Neurosciences, Institute of Neurology, UCL, Royal Free Campus, London, United Kingdom
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  • Bastiaan R. Bloem PhD,

    1. Department of Neurology, Donders Institute for Brain, Cognition and Behavior, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
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  • Philip D. Thompson PhD,

    1. Department of Neurology and University Department of Medicine, Royal Adelaide Hospital and University of Adelaide, Adelaide, South Australia
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  • Kailash Bhatia MD

    Corresponding author
    1. Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, UCL, London, United Kingdom
    • Sobell Department, Institute of Neurology, UCL, Queen Square, London WC1N 3BG, UK
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  • Relevant conflicts of interest/financial disclosures: Mark J. Edwards is supported by an NIHR Clinician Scientist Fellowship. Bastiaan R. Bloem is supported by the Netherlands Organization for Scientific Research (NWO; 016076352 and 92003490).

  • Full financial disclosures and author roles may be found in the online version of this article.

Abstract

Fixed dystonia is a disabling disorder mainly affecting young women who develop fixed abnormal limb postures and pain after apparently minor peripheral injury. There is continued debate regarding its pathophysiology and management. We report 5 cases of fixed dystonia in patients who sought amputation of the affected limb. We place these cases in the context of previous reports of patients with healthy limbs and patients with chronic regional pain syndrome who have sought amputation. Our cases, combined with recent data regarding disorders of mental rotation in patients with fixed dystonia, as well as previous data regarding body integrity identity disorder and amputations sought by patients with chronic regional pain syndrome, raise the possibility that patients with fixed dystonia might have a deficit in body schema that predisposes them to developing fixed dystonia and drives some to seek amputation. The outcome of amputation in fixed dystonia is invariably unfavorable. © 2011 Movement Disorder Society

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