Asayama Rie, Tanaka-Nishikubo Kaori, Iwanami Jun, Fukazawa Takahiro, Kanagawa Motoi, Hato Naohito
Department of Otolaryngology, Head, and Neck Surgery, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime, Japan.
Department of Cell Biology and Molecular Medicine, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime, Japan.
PLoS One. 2025 Sep 12;20(9):e0332511. doi: 10.1371/journal.pone.0332511. eCollection 2025.
Myotonic dystrophy is associated with dysphagia, which can lead to severe complications such as aspiration pneumonia and choking. However, few histopathological studies on dysphagia in myotonic dystrophy have been conducted. In this study, we aimed to validate the FVB/N-Tg(HSA*LR)20bCath mice for studying dysphagia associated with myotonic dystrophy, using videofluoroscopic swallowing study, histological analysis, and immunofluorescence analysis. Videofluoroscopic swallowing study, revealed significant abnormalities during the pharyngeal swallowing phase of swallowing in HSA LR20b mice, including increased pharyngeal residue area and prolonged pharyngeal transit time, suggesting that this mouse model was a valuable tool for studying dysphagia in myotonic dystrophy. These findings might represent a characteristic swallowing pattern in myotonic dystrophy. Histological analysis demonstrated marked variability in muscle fiber size and a high frequency of central nuclei. Additionally, decreased expression of chloride channel 1 was observed in the masseter muscle, suggesting the presence of myotonia. Collectively, these findings provide a foundation for further research into the complex mechanisms underlying myotonic dystrophy associated dysphagia and may inform the development of future treatment strategies.
强直性肌营养不良与吞咽困难有关，吞咽困难可导致严重并发症，如吸入性肺炎和窒息。然而，针对强直性肌营养不良患者吞咽困难的组织病理学研究却很少。在本研究中，我们旨在通过视频荧光吞咽造影研究、组织学分析和免疫荧光分析，验证FVB/N-Tg(HSA*LR)20bCath小鼠用于研究与强直性肌营养不良相关的吞咽困难。视频荧光吞咽造影研究显示，HSA LR20b小鼠在吞咽的咽部吞咽阶段存在明显异常，包括咽部残留面积增加和咽部通过时间延长，这表明该小鼠模型是研究强直性肌营养不良吞咽困难的有价值工具。这些发现可能代表了强直性肌营养不良的一种特征性吞咽模式。组织学分析显示肌纤维大小存在明显差异，且中央核的频率较高。此外，在咬肌中观察到氯离子通道1的表达降低，提示存在肌强直。总的来说，这些发现为进一步研究强直性肌营养不良相关吞咽困难的复杂机制奠定了基础，并可能为未来治疗策略的开发提供参考。
