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.
Cognitive impairment in adult myotonic dystrophies : a longitudinal study / V.A.M. Sansone, S. Gandossini, M. Cotelli, M. Calabria, O. Zanetti, G. Meola. - In: NEUROLOGICAL SCIENCES. - ISSN 1590-1874. - 28:1(2007 Mar), pp. 9-15.
|Titolo:||Cognitive impairment in adult myotonic dystrophies : a longitudinal study|
|Parole Chiave:||myotonic dystrophy type 1; myotonic dystrophy type 2; dysexecutive syndrome; follow-up; dementia|
|Settore Scientifico Disciplinare:||Settore MED/26 - Neurologia|
|Data di pubblicazione:||mar-2007|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1007/s10072-007-0742-z|
|Appare nelle tipologie:||01 - Articolo su periodico|