Event-related potentials using the auditory novel paradigm in patients with myotonic dystrophy.

Many neuropsychological disorders, especially attentional abnormality, are present in patients with myotonic dystrophy type 1 (DM1), but the underlying mechanisms remain unclear. This study aimed to evaluate attention function by auditory event-related potential (ERP) P3a (novelty paradigm) in DM1 patients. A total of 10 young DM1 patients (mean age 30.4 years) and 14 age-matched normal controls participated in this study. ERPs were recorded using an auditory novel paradigm, consisting of three types of stimuli, i.e., standard sound (70%), target sound (20%), and various novel sounds (10%), and participants pressed buttons to the target sounds. ERP components P3b after the target stimuli and P3a following the novel stimuli were analyzed. Correlations of neuropsychological evaluations with the amplitudes and latencies of P3b and P3a were analyzed in DM1 patients. We found that P3a latency was significantly delayed in patients with DM1 compared with normal controls, although the latency and amplitude of P3b in DM1 patients were comparable with those in normal controls. The achievement rates of both the Symbol Digit Modality Test and the Paced Auditory Serial Addition Test were significantly correlated with P3a amplitude, as well as P3b amplitude. These results suggest that ERPs, including P3a and P3b, provide important insights into the physiological basis of neuropsychological abnormalities in patients with DM1, especially from the viewpoint of the frontal lobe and attention function.