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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
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.
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.
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