Functional impairment in patients with myotonic dystrophy type 1 can be assessed by an ataxia rating scale (SARA) View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2017-02-06

AUTHORS

Giovanni DiPaolo, Cecilia Jimenez-Moreno, Nikoletta Nikolenko, Antonio Atalaia, Darren G. Monckton, Michela Guglieri, Hanns Lochmüller

ABSTRACT

Myotonic dystrophy type 1 (DM1) is not characterised by ataxia per se; however, DM1 and ataxia patients show similar disturbances in movement coordination often experiencing walking and balance difficulties, although caused by different underlying pathologies. This study aims to investigate the use of a scale previously described for the assessment and rating of ataxia (SARA) with the hypothesis that it could have utility in DM1 patients as a measure of disease severity and risk of falling. Data from 54 DM1 patients were pulled from the PHENO-DM1 natural history study for analysis. Mean SARA score in the DM1 population was 5.45 relative to the maximum score of eight. A flooring effect (score 0) was observed in mild cases within the sample. Inter-rater and test-retest reliability was high with intraclass coefficients (ICC) of 0.983 and 1.00, respectively. Internal consistency was acceptable as indicated by a Cronbach's alpha of 0.761. Component analysis revealed two principle components. SARA correlated with: (1) all measures of muscle function tested, including quantitative muscle testing of ankle dorsiflexion (r = -0.584*), the 6 min walk test (r = -0.739*), 10 m walk test (r = 0.741*), and the nine hole peg test (r = 0.602*) and (2) measures of disease severity/burden, such as MIRS (r = 0.718*), MDHI (r = 0.483*), and DM1-Activ (r = -0.749*) (*p < 0.001). The SARA score was predicted by an interaction between modal CTG repeat length and age at sampling (r = 0.678, p = 0.003). A score of eight or above predicted the use of a walking aid with a sensitivity of 100% and a specificity of 85.7%. We suggest that further research is warranted to ascertain whether SARA or components of SARA are useful outcome measures for clinical trials in DM1. As a tool, it can be used for gathering information about disease severity/burden and helping to identify patients in need of a walking aid, and can potentially be applied in both research and healthcare settings. More... »

PAGES

701-708

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s00415-017-8399-x

DOI

http://dx.doi.org/10.1007/s00415-017-8399-x

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1083688090

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/28168524


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85 healthcare settings
86 history studies
87 hypothesis
88 impairment
89 information
90 interaction
91 internal consistency
92 intraclass coefficient
93 length
94 maximum score
95 measures
96 mild cases
97 min
98 modal CTG repeat length
99 movement coordination
100 muscle function
101 muscle testing
102 myotonic dystrophy type 1
103 natural history studies
104 need
105 outcome measures
106 pathology
107 patients
108 population
109 principle components
110 quantitative muscle testing
111 ratings
112 reliability
113 repeat length
114 research
115 risk
116 samples
117 sampling
118 scale
119 scores
120 sensitivity
121 setting
122 severity
123 severity/burden
124 similar disturbances
125 specificity
126 study
127 test
128 test-retest reliability
129 testing
130 tool
131 trials
132 type 1
133 use
134 useful outcome measure
135 utility
136 walking
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