The Burke-Fahn-Marsden Dystonia Rating Scale is Age-Dependent in Healthy Children

Marieke Johanna Kuiper, Lois Vrijenhoek, Rick Brandsma, Roelineke J. Lunsing, Huibert Burger, Hendriekje Eggink, Kathryn J. Peall, Maria Fiorella Contarino, Johannes D. Speelman, Marina A. J. Tijssen, Deborah A. Sival*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

16 Citations (Scopus)
24 Downloads (Pure)

Abstract

Background: The Burke-Fahn-Marsden Dystonia Rating Scale is a universally applied instrument for the quantitative assessment of dystonia in both children and adults. However, immature movements by healthy young children may also show "dystonic characteristics" as a consequence of physiologically incomplete brain maturation. This could implicate that Burke-Fahn-Marsden scale scores are confounded by pediatric age.

Objective: In healthy young children, we aimed to determine whether physiologically immature movements and postures can induce an age-related effect on Burke-Fahn-Marsden movement and disability scale scores.

Methods: Nine assessors specializied in movement disorders (3 adult neurologists, 3 pediatric neurologists, and 3 MD/PhD students) independently scored the Burke-Fahn-Marsden movement scale in 52 healthy children (4-16 years of age; 2 boys and 2 girls per year of age). Independent of that, parents scored their children's functional motor development according to the Burke-Fahn-Marsden disability scale in another 52 healthy children (4-16 years of age; 2 boys and 2 girls per year of age). By regression analysis, we determined the association between Burke-Fahn-Marsden movement and disability scales outcomes and pediatric age.

Results: In healthy children, assessment of physiologically immature motor performances by the Burke-Fahn-Marsden movement and disability scales showed an association between the outcomes of both scales and age (until 16 years and 12 years of age, beta = -0.72 and beta = -0.60, for Burke-Fahn-Marsden movement and disability scale, respectively [both P <0.001]).

Conclusions: The Burke-Fahn-Marsden movement and disability scales are influenced by the age of the child. For accurate interpretation of longitudinal Burke-Fahn-Marsden Dystonia Rating Scale scores in young dystonic children, consideration of pediatric age-relatedness appears advisory.

Original languageEnglish
Pages (from-to)580-586
Number of pages7
JournalMovement Disorders Clinical Practice
Volume3
Issue number6
DOIs
Publication statusPublished - 2016

Keywords

  • Burke-Fahn-Marsden Dystonia Rating Scale
  • child
  • dystonia
  • validation
  • age
  • DEEP BRAIN-STIMULATION
  • MOVEMENT-DISORDERS
  • CHILDHOOD DYSTONIA
  • ATAXIA
  • RELIABILITY
  • CEREBELLUM
  • VALIDITY
  • UPDATE

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