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.
|Titolo:||Cognitive impairment in adult myotonic dystrophies : a londitudinal study|
|Autori interni:||MEOLA, GIOVANNI|
SANSONE, VALERIA ADA MARIA
|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:||2007|
|Digital Object Identifier (DOI):||10.1007/s10072-007-0742-z|
|Appare nelle tipologie:||01 - Articolo su periodico|
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