Dataset Information

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

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, β = -0.72 and β = -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.

SUBMITTER: Kuiper MJ

PROVIDER: S-EPMC6353340 | biostudies-literature | 2016 Nov-Dec

REPOSITORIES: biostudies-literature

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Publications

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

Kuiper Marieke Johanna MJ Vrijenhoek Loïs L Brandsma Rick R Lunsing Roelineke J RJ Burger Huibert H Eggink Hendriekje H Peall Kathryn J KJ Contarino Maria Fiorella MF Speelman Johannes D JD Tijssen Marina A J MAJ Sival Deborah A DA

Movement disorders clinical practice 20160503 6

<h4>Background</h4>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.<h4>Objective</h4>In healthy young children, we aimed to determine whether ph ...[more]

PMID: 30838251

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Project description:Background: Oromandibular dystonia manifests with sustained or task-specific contractions of the masticatory, tongue, and/or other muscles in the stomatognathic system. Since its symptoms can vary, it has been difficult to objectively measure disease severity and post-treatment changes. Objective: To develop and validate a comprehensive measurement tool for oromandibular dystonia. Methods: An examiner-rated scale included three subscales for severity, disability, and pain, modified specifically for oromandibular dystonia from the Toronto Western Spasmodic Torticollis Rating Scale-2. To evaluate the severity of each subtype of oromandibular dystonia, four of the six items were selected according to the subtype (jaw closing dystonia, tongue dystonia, jaw opening dystonia, jaw deviation [protrusion] dystonia, and lip dystonia). A patient-administered questionnaire based on clinical features and other relevant aspects associated with oromandibular dystonia was developed, which included five subscales: general, eating, speech, cosmetic, and social/family life. The questionnaire, examiner-rated scale, and four subscales (sleep, annoyance, mood, and psychosocial functioning) of the Cervical Dystonia Impact Profile-58 were combined to construct the oromandibular dystonia rating scale (OMDRS). The reliability and validity of the scale were assessed using clinimetric testing. Results: Six hundred and eighteen patients with oromandibular dystonia (394 women and 224 men; mean age, 51.7 years) were evaluated by the OMDRS. The overall OMDRS showed high-level internal consistency measured by Cronbach's alpha (0.95) with a logical factor structure. Cronbach's alpha for the subscales was satisfactory to excellent, ranging from 0.72 to 0.94. All items revealed acceptable inter-rater reliability (kappa > 0.4, interclass correlation coefficient > 0.6). Repeated ratings of videotapes revealed acceptable intra-rater reliability for all items (kappa > 0.76, interclass correlation coefficient > 0.86). Test-retest reliability showed a significant (p < 0.001) correlation efficiency. The OMDRS showed significant (p < 0.001) convergent and discriminant validity and significant (p < 0.001) sensitivity to changes after botulinum toxin therapy. Conclusion: The OMDRS can be useful for the comprehensive evaluation of disease severity, disability, psychosocial functioning, and impact on the quality of life as well as therapeutic changes in patients with oromandibular dystonia.

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Dataset Information

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

Background

Objective

Methods

Results

Conclusions

Publications

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

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