S. N. Bardakov, A. Polushin, V. A. Tsargush, P. Carlier, A. A. Emeliantsev, S. Lapin, A. Moshnikova, I. Skiba, A. Belskikh, A. Kulagin
{"title":"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","authors":"S. N. Bardakov, A. Polushin, V. A. Tsargush, P. Carlier, A. A. Emeliantsev, S. Lapin, A. Moshnikova, I. Skiba, A. Belskikh, A. Kulagin","doi":"10.30629/2658-7947-2022-27-4-44-54","DOIUrl":null,"url":null,"abstract":"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.","PeriodicalId":36724,"journal":{"name":"Russian Neurological Journal","volume":" ","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2022-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"1","resultStr":"{\"title\":\"Anti-MuSK myasthenia after allogeneic hematopoietic stem cell transplantation in acute myeloid leukemia\",\"authors\":\"S. N. Bardakov, A. Polushin, V. A. Tsargush, P. Carlier, A. A. Emeliantsev, S. Lapin, A. Moshnikova, I. Skiba, A. Belskikh, A. Kulagin\",\"doi\":\"10.30629/2658-7947-2022-27-4-44-54\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"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.\",\"PeriodicalId\":36724,\"journal\":{\"name\":\"Russian Neurological Journal\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2022-10-03\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"1\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Russian Neurological Journal\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.30629/2658-7947-2022-27-4-44-54\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Russian Neurological Journal","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.30629/2658-7947-2022-27-4-44-54","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
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.