Cognitive impairment in adult myotonic dystrophies: a longitudinal study |
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Authors: | V Sansone S Gandossini M Cotelli M Calabria O Zanetti G Meola |
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Institution: | (1) Department of Neurology, University of Milan IRCCS Policlinico San Donato, Via Morandi 30, I-20097 San Donato Milanese, Italy;(2) U.O. Alzheimer IRCCS S. Giovanni di Dio-FBF, Brescia, Italy;(3) Centro di Scienza Cognitiva, University of Torino, Italy |
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Abstract: | 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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Keywords: | Myotonic dystrophy type 1 Myotonic dystrophy type 2 Dysexecutive syndrome Follow-up Dementia |
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