Department of Child Development, United Graduate School of Child Development, Osaka University, Suita, Japan.
Graduate School of Human Sciences, Osaka University, Suita, Japan.
J Neuromuscul Dis. 2023;10(6):1083-1092. doi: 10.3233/JND-230083.
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.
This study aimed to describe longitudinal differences in neuropsychological function in patients with DM1.
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).
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).
These results suggest a cognitive decline in patients with DM1 and warrant further investigation into the possible effects of age-related changes.
1 型肌强直性营养不良（DM1）是一种肌肉疾病，可导致多种症状，包括中枢神经系统的症状。一些研究报告称 DM1 患者存在认知能力下降，但现有证据有限。
本研究旨在描述 DM1 患者神经心理学功能的纵向差异。
使用神经心理学成套测验对 66 例日本成年 DM1 患者进行调查，以评估包括记忆、加工速度和执行功能在内的多个认知领域。患者在基线后约 5 年（时间 1 和 2）进行神经心理学评估。
38 例患者进行了第二次神经心理学评估。时间 2 评估的参与者比未参加时间 2 评估的参与者年龄更小。与时间 1 相比，患者在时间 2 时出现了简易精神状态检查、连线测验（TMT）、方块设计和符号数字模态测验得分下降（P<0.05）。时间 1 时的年龄与 TMT-A 和 TMT-B 评分的下降相关（rho=0.57 和 0.45）。
这些结果表明 DM1 患者存在认知能力下降，需要进一步研究年龄相关变化的可能影响。
