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)To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Archives of Physical Medicine and RehabilitationAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Clinical presentation and early evolution of spastic ataxia of Charlevoix-Saguenay.Mov Disord. 2013; 28: 2011-2014
- Autosomal recessive spastic ataxia of Charlevoix-Saguenay: upper extremity aptitudes, functional independence and social participation.Int J Rehabil Res. 2004; 27: 253-256
- Autosomal recessive spastic ataxia of Charlevoix-Saguenay.Can J Neurol Sci. 1978; 5: 61-69
- Genetic epidemiology of autosomal recessive spastic ataxia of Charlevoix-Saguenay in northeastern Quebec.Genet Epidemiol. 1993; 10: 17-25
- ARSACS.in: Adam M.P. Ardinger H.H. Pagon R.A. GeneReviews® [Internet]. University of Washington, Seattle, Seattle (WA)2003: 8
- Recessive spastic ataxia of Charlevoix-Saguenay.in: Vinken P. Bruyn G. HL K. de Jong J. Handbook of clinical neurology hereditary neuropathies and spinocerebellar atrophies. North-Holland Pub. Co, Amsterdam1991: 451-459
- Recessive spastic ataxia of Charlevoix-Saguenay (RSACS): clinical, morphological and genetic studies.in: Lechtenberg R. Handbook of cerebellar diseases. Marcel Dekker, New York1993: 491-494
- Measurement in medicine: a practical guide.Cambridge University Press, United Kingdom2011
- COSMIN checklist manual.(Available at:) (Accessed September 1, 2017)
- Adult norms for the Nine Hole Peg Test of finger dexterity.OTJR (Thorofare N J). 1985; 5: 24-38
- Neurology.Charles C. Thomas, Springfield1966
- International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neurology.J Neurol Sci. 1997; 145: 205-211
- Scale for the assessment and rating of ataxia: development of a new clinical scale.Neurology. 2006; 66: 1717-1720
- Clinical assessment recommendations.2nd ed. American Society of Hand Therapist, Chicago1992
- Intraclass correlations: uses in assessing rater reliability.Psychol Bull. 1979; 86: 420-428
- Foundations of clinical research: applications to practice.2nd ed. Prentice Hall Health, New Jersey2000
- Autosomal recessive spastic ataxia of Charlevoix Saguenay (ARSACS): expanding the genetic, clinical and imaging spectrum.Orphanet J Rare Dis. 2013; 8: 41
Article info
Publication history
Published online: February 17, 2018
Footnotes
Supported by the European Union’s Horizon 2020 research and innovation program under the ERA-NET Cofund action (award no. 6435780); the CIFR team (Muscular Dystrophy Canada and Ataxia of Charlevoix-Saguenay Foundation); the Ataxia of Charlevoix-Saguenay Foundation; BMBF (grant no. 01GM1607), under the frame of the E-Rare-3 network PREPARE; the Canadian Institutes of Health Research in partnership with Ataxia of Charlevoix-Saguenay Foundation (emerging team grant no. TR2-119189); and career grant funding from Fonds de recherche en santé du Québec (grant nos. 22193 and 31011).
Disclosures: Synofzik reports a financial relation with Actelion Pharmaceuticals, outside the submitted work. The other authors have nothing to disclose.
Identification
Copyright
© 2017 by the American Congress of Rehabilitation Medicine
