BACKGROUND Immune-mediated necrotizing myopathy is a rare autoimmune myopathy characterized by muscle weakness and elevated serum creatine kinase,with unique skeletal muscle pathology and magnetic resonance imaging fe...BACKGROUND Immune-mediated necrotizing myopathy is a rare autoimmune myopathy characterized by muscle weakness and elevated serum creatine kinase,with unique skeletal muscle pathology and magnetic resonance imaging features.CASE SUMMARY In this paper,two patients are reported:One was positive for anti-signal recognition particle antibody,and the other was positive for anti-3-hydroxy-3-methylglutaryl coenzyme A reductase antibody.CONCLUSION The clinical characteristics and treatment of the two patients were analysed,and the literature was reviewed to improve the recognition,diagnosis,and treatment of this disease.展开更多
Background： Clinically, it is difficult to differentiate multiple acyl-CoA dehydrogenase deficiency （MADD） from immune-mediated necrotizing myopathy （IMNM） because they display similar symptoms. This study aimed ...Background： Clinically, it is difficult to differentiate multiple acyl-CoA dehydrogenase deficiency （MADD） from immune-mediated necrotizing myopathy （IMNM） because they display similar symptoms. This study aimed to determine whether muscle magnetic resonance imaging （MRI） could be used for differential diagnosis between MADD and IMNM. Methods： The study evaluated 25 MADD patients, confirmed by muscle biopsy and ETFDH gene testing, and 30 IMNM patients, confirmed by muscle biopsy. Muscles were assessed for edema and fatty replacement using thigh MRI （tMRI）. Degrees and distribution patterns of fatty infiltration and edema in gluteus maximus and thigh muscles were compared. Results： Total fatty infiltration and edema scores （median, [Q 1, Q3]） were 4.00 （1.00, 15.00） and 0 （0, 4.00） in MADD and 14.50 （8.00, 20.75） and 22.00 （16.75, 32.00） in IMNM, respectively, which were significantly more severe in IMNM than that in MADD （P = 0.000 and P = 0.004~ respectively）. Edema scores tbr gluteus maximus, long head of biceps femoris, and semimembranosus were significantly higher in IMNM than in MADD （all P = 0.000）. Fatty infiltration scores for anterior and medial compartments were significantly more severe in IMNM than that in MADD （all P = 0.000）. Conclusion： Different patterns of muscle involvement on tMRI can contribute to differential diagnosis between MADD and IMNM when clinical suspicions alone are insufficient, thereby reducing the need for muscle biopsy.展开更多
文摘BACKGROUND Immune-mediated necrotizing myopathy is a rare autoimmune myopathy characterized by muscle weakness and elevated serum creatine kinase,with unique skeletal muscle pathology and magnetic resonance imaging features.CASE SUMMARY In this paper,two patients are reported:One was positive for anti-signal recognition particle antibody,and the other was positive for anti-3-hydroxy-3-methylglutaryl coenzyme A reductase antibody.CONCLUSION The clinical characteristics and treatment of the two patients were analysed,and the literature was reviewed to improve the recognition,diagnosis,and treatment of this disease.
文摘Background： Clinically, it is difficult to differentiate multiple acyl-CoA dehydrogenase deficiency （MADD） from immune-mediated necrotizing myopathy （IMNM） because they display similar symptoms. This study aimed to determine whether muscle magnetic resonance imaging （MRI） could be used for differential diagnosis between MADD and IMNM. Methods： The study evaluated 25 MADD patients, confirmed by muscle biopsy and ETFDH gene testing, and 30 IMNM patients, confirmed by muscle biopsy. Muscles were assessed for edema and fatty replacement using thigh MRI （tMRI）. Degrees and distribution patterns of fatty infiltration and edema in gluteus maximus and thigh muscles were compared. Results： Total fatty infiltration and edema scores （median, [Q 1, Q3]） were 4.00 （1.00, 15.00） and 0 （0, 4.00） in MADD and 14.50 （8.00, 20.75） and 22.00 （16.75, 32.00） in IMNM, respectively, which were significantly more severe in IMNM than that in MADD （P = 0.000 and P = 0.004~ respectively）. Edema scores tbr gluteus maximus, long head of biceps femoris, and semimembranosus were significantly higher in IMNM than in MADD （all P = 0.000）. Fatty infiltration scores for anterior and medial compartments were significantly more severe in IMNM than that in MADD （all P = 0.000）. Conclusion： Different patterns of muscle involvement on tMRI can contribute to differential diagnosis between MADD and IMNM when clinical suspicions alone are insufficient, thereby reducing the need for muscle biopsy.
