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Complex regional pain syndrome with dystonia in childhood


We present 6 children with Complex regional pain syndrome (CRPS) in whom dystonia, including abnormal posture and involuntary movement was a prominent feature. CRPS is known to occur in childhood usually involving severe limb pain, colour and temperature change, with associated loss of function. Dystonia associated with CRPS has never been described in children, although a few cases in adults have been reported. Here we review the presentation, diagnostic implications, management and prognosis.

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A retrospective case note review.


Six children (5 F, 1 M ; age range 11–18 years) with CRPS were identified as having dystonia. The initial presenting limb was the foot in all cases, although all 4 limbs were included in 1 instance. The degree of dystonia varied from mild shaking at rest to extreme and violent movement of the whole limb increasing with touch. Although the other features of CRPS were also present, including mechanical and thermal allodynia and temperature changes, the presence of dystonia led to diagnostic confusion. Management of these cases was also difficult and protracted but in the majority some improvement was obtained using multidisciplinary management. Physiotherapy was the most successful intervention.


Dystonia has not previously been recognised as a feature of CRPS in children and may confound diagnosis. Children with CRPS can develop abnormal movement and this is often linked to a more severe and complex presentation requiring a very intensive rehabilitation approach.

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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Maillard, S., Howard, R. & Hasson, N. Complex regional pain syndrome with dystonia in childhood. Pediatr Rheumatol 6 (Suppl 1), P170 (2008).

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