Longitudinal Changes in Neuropsychological Functioning in Japanese Patients with Myotonic Dystrophy Type 1: A Five Year Follow-Up Study

Author:

Fujino Haruo12,Suwazono Shugo3,Ueda Yukihiko4,Kobayashi Michio5,Nakayama Takahiro6,Imura Osamu7,Matsumura Tsuyoshi8,Takahashi Masanori P.910

Affiliation:

1. Department of Child Development, United Graduate School of Child Development, Osaka University, Suita, Japan

2. Graduate School of Human Sciences, Osaka University, Suita, Japan

3. Center for Clinical Neuroscience, National Hospital Organization Okinawa National Hospital, Ginowan, Japan

4. Okinawa International University, Ginowan, Japan

5. Department of Neurology, National Hospital Organization Akita National Hospital, Yurihonjo, Japan

6. Department of Neurology, Yokohama Rosai Hospital, Yokohama, Japan

7. Faculty of Social Sciences, Nara University, Nara, Japan

8. Department of Neurology, National Hospital Organization Osaka Toneyama Medical Center, Toyonaka, Japan

9. Department of Clinical Laboratory and Biomedical Sciences, Division of Health Sciences, Osaka University Graduate School of Medicine, Suita, Japan

10. Department of Neurology, Osaka University Graduate School of Medicine, Suita, Japan

Abstract

Background: Myotonic dystrophy type 1 (DM1) is a form of muscular dystrophy that causes various symptoms, including those of the central nervous system. Some studies have reported cognitive decline in patients with DM1, although the available evidence is limited. Objective: This study aimed to describe longitudinal differences in neuropsychological function in patients with DM1. Methods: A total of 66 Japanese adult patients with DM1 were investigated using a neuropsychological battery to assess several cognitive domains, including memory, processing speed, and executive function. The patients underwent neuropsychological evaluation approximately five years after baseline (Times 1 and 2). Results: Thirty-eight patients underwent a second neuropsychological evaluation. The participants in the Time 2 evaluation were younger than those who did not participate in Time 2. Patients showed a decline in the Mini-Mental State Examination, Trail Making Test (TMT), Block Design, and Symbol Digit Modalities Test at Time 2 (P < 0.05). Age at Time 1 was associated with a decline in TMT-A and TMT-B scores (rho = 0.57 and 0.45, respectively). Conclusion: These results suggest a cognitive decline in patients with DM1 and warrant further investigation into the possible effects of age-related changes.

Publisher

IOS Press

Subject

Neurology (clinical),Neurology

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