title: Limb amputations in fixed dystonia: a form of body integrity identity disorder?
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.
authors.affiliation: Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, UCL, London, UK.
authors.forename: Mark J
authors.initials: MJ
authors.lastname: Edwards
authors.forename: Araceli
authors.initials: A
authors.lastname: Alonso-Canovas
authors.forename: Arnette
authors.initials: A
authors.lastname: Schrag
authors.forename: Bastiaan R
authors.initials: BR
authors.lastname: Bloem
authors.forename: Philip D
authors.initials: PD
authors.lastname: Thompson
authors.forename: Kailash
authors.initials: K
authors.lastname: Bhatia
mesh_terms: D000328:Adult; D000671:Amputation, Surgical / Q000009:adverse effects / Q000379:methods*; D004192:Discrimination, Psychological / Q000502:physiology*; D004421:Dystonia / Q000523:psychology* / Q000601:surgery*; D005121:Extremities / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010146:Pain / Q000150:complications; D010468:Perceptual Disorders / Q000209:etiology*; D014947:Wounds and Injuries / Q000150:complications / Q000503:physiopathology