Neurocognitive disorder in Myotonic dystrophy type 1

Area of Science:

• Neurology
• Genetics
• Cognitive Science

Background:

• Myotonic dystrophy type 1 (DM1) is linked to cognitive deficits and aging.
• The progression and extent of cognitive decline in DM1 are not well understood.

Purpose of the Study:

• To investigate the prevalence of neurocognitive disorders (mild cognitive impairment and dementia) in adult DM1 patients.
• To assess cognitive function across different DM1 onset types.

Main Methods:

• A screening of 128 adult DM1 patients using the Montreal Cognitive Assessment (MoCA).
• Data collection included demographic and clinical information.
• Patients were categorized by DM1 onset: childhood, juvenile, adult, and late onset.

Main Results:

• Neurocognitive disorder signs were infrequent overall.
• 23.8% of late-onset DM1 patients (over 60) scored below the MoCA threshold.
• Late-onset DM1 patients performed significantly worse than adult-onset patients.
• Age at examination showed a weak negative correlation with MoCA scores.

Conclusions:

• Adult DM1 patients generally exhibit a low prevalence of severe neurocognitive disorders.
• Late-onset DM1 warrants further investigation due to observed cognitive differences.
• Longitudinal studies with larger samples are recommended to explore DM1 cognitive progression.