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  • Title: Cognitive impairment in adult myotonic dystrophies: a longitudinal study.
    Author: Sansone V, Gandossini S, Cotelli M, Calabria M, Zanetti O, Meola G.
    Journal: Neurol Sci; 2007 Mar; 28(1):9-15. PubMed ID: 17385090.
    Abstract:
    The clinical relevance and extent of cognitive impairment in adult myotonic dystrophy type 1 (DM1) and 2 (DM2) is still unclear. The aim of this study was to determine whether previously reported cognitive abnormalities progress over time and if this occurs in DM2 as it does in DM1. Fifty-six patients with DM1 and 29 patients with DM2 were subjected to muscle strength assessment, and to a complete battery of neuropsychological tests. Repeated assessment was performed in 20 DM1 and 13 DM2 over time (DM1 mean follow-up: 7.3+/-2.7 years; DM2 mean follow- up: 9.5+/-2.4 years). Muscle strength and test scores for frontal lobe functions worsened significantly over time (p<0.01), in both DM1 and DM2. DM2 is a progressive muscle disorder, although less severe than DM1. In both DM1 and DM2 frontal cognitive impairment (attentional) worsens over time but does not extend to additional areas of cognition.
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