急性髓系白血病同种异体造血干细胞移植后的抗麝香肌无力

Q4 Medicine
S. N. Bardakov, A. Polushin, V. A. Tsargush, P. Carlier, A. A. Emeliantsev, S. Lapin, A. Moshnikova, I. Skiba, A. Belskikh, A. Kulagin
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引用次数: 1

摘要

抗麝香肌无力是一种因agrin-MuSK-Lrp4复合物功能被破坏而引起的慢性自身免疫性疾病,常伴有病理性肌肉疲劳的发展,常伴有肌肉萎缩。这种疾病可以是原发性的,也可以是免疫抑制治疗和造血干细胞移植的并发症。我们报告了一例44岁(1975年出生)的急性髓系白血病患者在同种异体造血干细胞移植后发生的抗musk重症肌无力。该病例的一个显著特征是广泛性损伤,不仅包括眼外肌,而且包括椎旁肌,晚上出现低垂头综合征。此外,实验室、神经肌电图和神经成像(肌肉MRI、磁共振光谱)数据表明,所涉及的肌肉组织的变化具有神经源性可逆性。本研究报告了一名因抗musk重症肌无力而出现椎旁肌肉无力和肿胀(STIR显示水肿变化,对应于早期功能性去神经的MR征象),持续2-4个月的轴向肌肉神经源性损伤的MRI和EMG征象。经病理治疗后,临床表现及椎旁肌肉水肿性神经源性改变均有所消退。结论。文章介绍了重症肌无力的临床和仪器特征的最新数据。所考虑的临床例子证实了抗musk重症肌无力早期存在神经源性改变,提示需要立即开始病理治疗,以避免肌肉萎缩和脂肪浸润的发展。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Anti-MuSK myasthenia after allogeneic hematopoietic stem cell transplantation in acute myeloid leukemia
Anti-MuSK myasthenia is a chronic autoimmune disease caused by violation of the function of the agrin-MuSK-Lrp4 complex, accompanied by the development of pathological muscle fatigue and often muscle atrophy. The disease can be a primary, as well as a complication of immunosuppressive therapy and hematopoietic stem cell transplantation. We present a case of anti-MUSK myasthenia gravis that developed after allogeneic HSCT due to acute myeloid leukemia in a 44-year-old patient (born in 1975). A distinctive feature of the case was a generalized injury, including not only extraocular, but also paravertebral muscles, dropped head syndrome in the evening. In addition, laboratory, electroneuromyographic and neuroimaging (MRI of muscles, MR-spectroscopy) data indicating the neurogenic reversible nature of changes in the involved musculature are presented.Results. This study presents MRI and EMG signs of neurogenic damage of axial musculature in a patient with weakness and swelling of the paravertebral muscles (edematous changes according to STIR, corresponding to MR signs of early functional denervation) for a period of 2–4 months, due to anti-MUSK myasthenia gravis. Clinical manifestations, as well as edematous neurogenic changes in the paravertebral muscles, regressed after pathogenetic therapy. Conclusion. The article presents up-to-date data on the clinical and instrumental characteristics of myasthenia gravis. The considered clinical example confirms the presence of neurogenic changes at an early stage of anti-MUSK myasthenia gravis and indicates the need for immediate initiation of pathogenetic therapy to avoid the development of atrophy and fatty infiltration of muscles.
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来源期刊
Russian Neurological Journal
Russian Neurological Journal Medicine-Neurology (clinical)
CiteScore
0.40
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发文量
49
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