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Validity and Reliability of Outcome Measures Assessing Dexterity, Coordination, and Upper Limb Strength in Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay

  • Cynthia Gagnon
    Correspondence
    Corresponding author Cynthia Gagnon, OT, PhD, GRIMN, CIUSSS du Saguenay-Lac-St-Jean, site Jonquière, 2230 de l’Hôpital, C.P. 1200, Jonquière (Québec) Canada, G7X 7X2.
    Affiliations
    Charles-Le-Moyne-Saguenay-Lac-St-Jean Research Center, Faculty of Medicine and Health Sciences, University of Sherbrooke, QC, Canada

    Groupe de Recherche Interdisciplinaire Sur Les Maladies Neuromusculaires, Centre Intégré Universitaire de Santé et de Services Sociaux du Saguenay-Lac-St-Jean, QC, Canada
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  • Isabelle Lessard
    Affiliations
    Charles-Le-Moyne-Saguenay-Lac-St-Jean Research Center, Faculty of Medicine and Health Sciences, University of Sherbrooke, QC, Canada

    Groupe de Recherche Interdisciplinaire Sur Les Maladies Neuromusculaires, Centre Intégré Universitaire de Santé et de Services Sociaux du Saguenay-Lac-St-Jean, QC, Canada
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  • Bernard Brais
    Affiliations
    Montreal Neurological Institute, McGill University, QC, Canada
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  • Isabelle Côté
    Affiliations
    Groupe de Recherche Interdisciplinaire Sur Les Maladies Neuromusculaires, Centre Intégré Universitaire de Santé et de Services Sociaux du Saguenay-Lac-St-Jean, QC, Canada
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  • Caroline Lavoie
    Affiliations
    Groupe de Recherche Interdisciplinaire Sur Les Maladies Neuromusculaires, Centre Intégré Universitaire de Santé et de Services Sociaux du Saguenay-Lac-St-Jean, QC, Canada
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  • Matthis Synofzik
    Affiliations
    Department of Neurodegeneration, Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany

    German Center for Neurodegenerative Diseases, Tübingen, Germany
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  • Jean Mathieu
    Affiliations
    Groupe de Recherche Interdisciplinaire Sur Les Maladies Neuromusculaires, Centre Intégré Universitaire de Santé et de Services Sociaux du Saguenay-Lac-St-Jean, QC, Canada
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Published:February 17, 2018DOI:https://doi.org/10.1016/j.apmr.2018.01.026

      Abstract

      Objective

      To document in adults affected by autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) the intra- and interrater reliability, standard error of measurement, agreement, minimal detectable change, and construct validity of the 9-Hole Peg Test (NHPT), the Standardized Finger-to-Nose Test (SFNT), and grip strength.

      Design

      Metrologic study.

      Setting

      Neuromuscular rehabilitation clinic.

      Participants

      Genetically confirmed adult patients with ARSACS (N=42; 21 women; mean age, 38.6y).

      Interventions

      Not applicable.

      Main Outcome Measures

      Intra- and interrater reliability was determined using the intraclass correlation coefficient (ICC). Construct validity was determined by assessing the capacity of the NHPT, the SFNT, and grip strength to distinguish between participants based on sex, mobility stages, and age groups, and on performance on the Archimedes spiral and fast alternating hand movements tests.

      Results

      All 3 tests have shown excellent reliability (ICC=.90–.98). However, the limit of agreement was influenced by the participant’s performance on the NHPT, and the minimal detectable change was very different for both hands (right=9.7 vs left=28.0). Construct validity was confirmed for the SFNT and NHPT, but it was not demonstrated for grip strength.

      Conclusions

      Given the metrologic properties assessed in this study, the SFNT is an excellent measure to assess upper limb coordination, whereas the NHPT must be used with caution. The grip strength is reliable but does not seem to reflect disease severity.

      Keywords

      List of abbreviations:

      ARSACS (autosomal recessive spastic ataxia of Charlevoix-Saguenay), ICC (intraclass correlation coefficient), MDC (minimal detectable change), NHPT (9-Hole Peg Test), SFNT (Standardized Finger-to-Nose Test)
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