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Brief assessment of cognitive function in myotonic dystrophy: multicenter longitudinal study using computer-assisted evaluation. Muscle & nerve Deutsch, G. K., Hagerman, K. A., Sampson, J., Dent, G., Dekdebrun, J., Parker, D. M., Thornton, C. A., Heatwole, C. R., Subramony, S. H., Mankodi, A. K., Ashizawa, T., Statland, J. M., Arnold, W. D., Moxley, R. T., Day, J. W. 2022

Abstract

Myotonic dystrophy type 1 (DM1) is known to affect cognitive function, but the best methods to assess CNS involvement in multicenter studies have not been determined. This study's primary aim was to evaluate the potential of computerized cognitive tests to assess cognition in DM1.We conducted a prospective, longitudinal, observational study of 113 adults with DM1 at 6 sites. Psychomotor speed, attention, working memory, and executive functioning were assessed at baseline, 3-months and 12-months using computerized cognitive tests. Results were compared with assessments of muscle function and patient reported outcomes (PROs), including the Myotonic Dystrophy Health Index (MDHI) and EQ-5D-5L.Based on intra-class correlation coefficients (ICCs), computerized cognitive tests had moderate to good reliability for psychomotor speed (0.76), attention (0.82), working memory speed (0.79), working memory accuracy (0.65), and executive functioning (0.87). Performance at baseline was lowest for working memory accuracy (p 

View details for DOI 10.1002/mus.27520

View details for PubMedID 35179228