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Progression to Musculoskeletal Deformity in Childhood Dystonia

Research output: Contribution to journalArticle

Original languageEnglish
JournalEuropean Journal of Paediatric Neurology
Early online date19 Feb 2016
DOIs
Accepted/In press7 Feb 2016
E-pub ahead of print19 Feb 2016

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    1_s2.0_S1090379816000398_main.pdf, 818 KB, application/pdf

    Uploaded date:19 Feb 2016

    Version:Accepted author manuscript

King's Authors

Abstract

AbstractAim Dystonia is a movement disorder characterized by involuntary muscle contractions, resulting in abnormalities of posture and movement. Children with dystonia are at risk of developing fixed musculoskeletal deformities (FMDs). FMDs cause pain, limit function and participation and interfere with care. We aimed to explore factors relating to the development of FMD in a large cohort of children with dystonia. Method The case notes of all children referred to our Complex Motor Disorder service between July 2005 and December 2011 were reviewed. Data from 279 children (median age 9 years 10 months, Standard Deviation 4 years 2 months) with motor disorders including a prominent dystonic element were analysed. Parametric accelerated failure time regression was used to identify the factors related to development of contractures. Results FMDs were present at referral in more than half (n=163, 58%) of cases. Three quarters (n=120, 74%) of children with FMD had deformities around the hip, and 42% had spinal deformity (n=68). Compared to pure primary dystonia, FMD onset was earlier with a diagnosis of secondary or heredodegenerative dystonia, and a mixed spastic-dystonic phenotype (all p<0.001). FMD onset was also earlier with increasing Gross Motor Function Classification System (GMFCS) level (p<0.001). The effect of aetiological classification was lost when controlling for GMFCS level and motor phenotype. Interpretation Children with secondary or heredodegenerative dystonia are at greater risk of progression to FMD compared to primary dystonia, likely due to more severe dystonia within these groups. Children with additional spasticity are at particular risk, requiring close monitoring.

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