Abstract
Aim: Myotonic dystrophy type 1 (DM1) is a multisystem progressive neuromuscular disorder, characterized by myotonia and muscle weakness, usually accompanied by other systemic involvements such as cardiac or endocrine systems. Central nervous system involvement includes subtle to severe cognitive decline, somnolence and apathia. In this study, we aimed to demonstrate the cognitive abnormalities in DM1 patients using event-related potentials (P300). Material and Method: A total of 13 patients diagnosed as DM1 in Neuromuscular Unit in TCSB Izmir Tepecik Research and Training Hospital and 8 healthy control individuals were included in the study. Demographic features of patients such as age, sex and education information were gathered from the registry retrospectively. Event-related P300 potentials were recorded using standard two-tone auditory “oddball” paradigm. Findings: Although there was no difference between the patients and controls in regard of age, gender and education, P300 potentials were absent in 2 patients and statistically significantly delayed in the rest of 11 patients compared to the controls (p<0,001). Conclusion: This is a preliminary study. Our findings demonstrated the cognitive abnormalities in DM1 patients in consis-tency with the previous studies. We know that subtle to severe cognitive decline is a major concern in DM1. Event-related auditory potentials (P300), independent of motor functions, could be safely used in the follow-up of these patients.
Keywords: Myotonic dystrophy type I, Cognition, Cognitive decline, P300, Event-related potentials
