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肌肉特异性酪氨酸激酶抗体阳性重症肌无力的长期吞咽康复:一例报告

Long-Term Swallowing Rehabilitation for Muscle-Specific Tyrosine Kinase Antibody-Positive Myasthenia Gravis: A Case Report.

作者信息

Oshima Yuki, Nanto Tomoki, Eimoto Kazuki, Uchiyama Yuki, Domen Kazuhisa

机构信息

Department of Rehabilitation, Hyogo Medical University Hospital, Nishinomiya, JPN.

Department of Speech-Language-Hearing Therapy, Faculty of Rehabilitation, Morinomiya University of Medical Sciences, Osaka, JPN.

出版信息

Cureus. 2025 Mar 13;17(3):e80544. doi: 10.7759/cureus.80544. eCollection 2025 Mar.

DOI:10.7759/cureus.80544
PMID:40225522
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11993821/
Abstract

Muscle-specific tyrosine kinase antibody-positive myasthenia gravis (MuSK-MG) often presents with severe dysphagia. However, effective strategies for swallowing rehabilitation in these patients have not been well established. We report a case of a MuSK-MG patient with severe dysphagia who underwent swallowing rehabilitation for approximately 12 months alongside drug therapy and showed improvement in swallowing function. The patient was a 67-year-old woman treated with high-dose intravenous methylprednisolone pulse therapy, high-dose intravenous immunoglobulin, and plasma exchange (PE) therapy. She required ventilator management and tracheotomy. The patient subsequently developed recurrent pneumonia, and rituximab was administered due to a poor response to previous treatments. Although the primary disease had apparently stabilized, the patient continued to experience severe dysphagia with silent aspiration, delayed swallowing reflexes, and pharyngeal residues. To address these issues, intensive and prolonged swallowing rehabilitation was implemented, including regular swallowing assessments, progressive muscle-strengthening exercises with controlled load levels, and interferential current stimulation to address sensory disturbances. Consequently, the patient successfully achieved oral intake without experiencing a myasthenic crisis or recurrence of aspiration pneumonia. These findings suggest that long-term swallowing rehabilitation, combined with appropriate treatment of the underlying disease, may effectively improve swallowing function in patients with MuSK-MG and persistent severe dysphagia.

摘要

肌肉特异性酪氨酸激酶抗体阳性重症肌无力(MuSK-MG)常伴有严重吞咽困难。然而,针对这些患者吞咽功能康复的有效策略尚未完全确立。我们报告一例MuSK-MG伴严重吞咽困难的患者,该患者在药物治疗的同时接受了约12个月的吞咽功能康复治疗,吞咽功能得到改善。患者为一名67岁女性,接受了大剂量静脉注射甲基强的松龙冲击治疗、大剂量静脉注射免疫球蛋白和血浆置换(PE)治疗。她需要呼吸机管理和气管切开术。该患者随后出现复发性肺炎,因对先前治疗反应不佳而给予利妥昔单抗治疗。尽管原发性疾病明显稳定,但患者仍持续存在严重吞咽困难,伴有隐性误吸、吞咽反射延迟和咽部残留。为解决这些问题,实施了强化和长期的吞咽功能康复治疗,包括定期吞咽评估、在控制负荷水平下进行渐进性肌肉强化锻炼以及针对感觉障碍的干扰电流刺激。结果,患者成功实现经口进食,未发生肌无力危象或误吸性肺炎复发。这些发现表明,长期吞咽功能康复结合对基础疾病的适当治疗,可能有效改善MuSK-MG伴持续性严重吞咽困难患者的吞咽功能。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/39b8/11993821/133acc7744e1/cureus-0017-00000080544-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/39b8/11993821/425cc81dc9d0/cureus-0017-00000080544-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/39b8/11993821/133acc7744e1/cureus-0017-00000080544-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/39b8/11993821/425cc81dc9d0/cureus-0017-00000080544-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/39b8/11993821/133acc7744e1/cureus-0017-00000080544-i02.jpg

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