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Facial memory deficits in myotonic dystrophy type 1.

J L Kleberg1, C Lindberg, S Winblad

  • 1Department of Psychology, University of Gothenburg, Gothenburg, Sweden; Department of Psychology, Uppsala University, Uppsala, Sweden.

Acta Neurologica Scandinavica
|February 18, 2014
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Summary

Patients with myotonic dystrophy type 1 (DM1) show deficits in facial memory ability (FMA). This impairment is linked to poorer visual construction and memory skills, but not disease factors like CTG repeat size.

Keywords:
CTG repeatscognitionfacial memorymyotonic dystrophy type 1

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Area of Science:

  • Neuroscience
  • Genetics
  • Neurology

Background:

  • Myotonic dystrophy type 1 (DM1) is a multisystem disorder.
  • Cognitive impairments, including memory deficits, are common in DM1.
  • Facial memory ability (FMA) has not been extensively studied in DM1.

Purpose of the Study:

  • To assess FMA in patients with DM1.
  • To investigate the correlation between FMA and neuropsychological performance.
  • To explore the relationship between FMA and disease-related factors, including CTG repeat expansion size.

Main Methods:

  • A cohort of 33 DM1 patients and 30 healthy controls were evaluated.
  • Facial memory ability was assessed using the Rivermead Behavioural Memory Test - Extended version (RBMT-E) faces task.
  • Neuropsychological tests, clinical data collection, and CTG repeat size quantification were performed.

Main Results:

  • DM1 patients exhibited significantly lower FMA compared to controls (P < 0.05).
  • 36% of DM1 patients demonstrated impaired FMA.
  • FMA deficits correlated with poorer visual-construction and memory abilities and increased false recognition of unfamiliar faces.
  • No association was found between FMA deficits and disease-related factors, including CTG repeat size.

Conclusions:

  • Individuals with DM1 display notable deficits in facial memory ability.
  • These FMA impairments in DM1 are associated with reduced visual construction and memory capacities.
  • FMA deficits in DM1 are independent of disease duration or CTG repeat expansion length.