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

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Abstract

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.

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Acknowledgements

We appreciate the technical support by Ms. Megumi Okuma and Mr. Naoki Yoshimura. We thank Editage (http://www.editage.com) for English language editing.

Funding

This study was supported by Grant-in-Aid for the Japan Agency for Medical Research and Development (JP19ek0109259 and JP20ek0109474) and Grant-in-Aid for Scientific Research KAKENHI (C) (18K11406 and 20K00539).

Author information

Authors and Affiliations

  1. Department of Neurology and Center for Clinical Neuroscience, National Hospital Organization Okinawa National Hospital, 3-20-14 Ganeko, Ginowan, 901-2214, Japan

    Shugo Suwazono

  2. Department of Human Sciences, Taisho University, Tokyo, Japan

    Hiroshi Arao

  3. Department of Human Welfare, Okinawa International University, Ginowan, Japan

    Yukihiko Ueda & Shino Maedou

Authors
  1. Shugo Suwazono
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  2. Hiroshi Arao
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  3. Yukihiko Ueda
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  4. Shino Maedou
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Contributions

SS, MD, PhD: study concept, design, and ERP data collection and analysis, and writing the first draft; HA, PhD: study design, data interpretation, and manuscript editing; YU, PhD: data interpretation and manuscript editing; SM, MPs: data interpretation and manuscript editing.

Corresponding author

Correspondence to Shugo Suwazono.

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Conflicts of interest

The authors have no conflicts of interest to declare.

Ethics approval

The present study was approved by the institutional review board of the National Hospital Organization Okinawa National Hospital (#25-03, #26-23). The procedures used in this study adhered to the tenets of the Declaration of Helsinki. All the participants signed informed consent before attending the recording of ERPs.

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Informed consent was obtained from all individual participants included in the study.

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Suwazono, S., Arao, H., Ueda, Y. et al. Event-related potentials using the auditory novel paradigm in patients with myotonic dystrophy. J Neurol 268, 2900–2907 (2021). https://doi.org/10.1007/s00415-021-10465-1

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