Cognitive impairment in adult myotonic dystrophies: A longitudinal study

V. Sansone, S. Gandossini, M. Cotelli, M. Calabria, O. Zanetti, Giovanni Meola

Research output: Contribution to journalArticlepeer-review


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

Original languageEnglish
Pages (from-to)9-15
Number of pages7
JournalNeurological Sciences
Issue number1
Publication statusPublished - Mar 2007


  • Dementia
  • Dysexecutive syndrome
  • Follow-up
  • Myotonic dystrophy type 1
  • Myotonic dystrophy type 2

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology


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