Park Sunha, Jang Dae-Hyun, Kim Jae-Min, Yoon Nara
Department of Rehabilitation Medicine, Incheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea.
Department of Pathology, Incheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea.
Diagnostics (Basel). 2021 Nov 8;11(11):2064. doi: 10.3390/diagnostics11112064.
Immune-mediated necrotizing myopathy, a new subgroup of inflammatory myopathies, usually begins with subacute onset of symmetrical proximal muscle weakness. A 35-year-old male presented with severe asymmetric iliopsoas atrophy and low back pain with a previous history of left lower extremity weakness. Although his first left lower extremity weakness occurred 12 years ago, he did not receive a clear diagnosis. Magnetic resonance imaging of both thigh muscles showed muscle edema and contrast enhancement in patch patterns, and the left buttock and thigh muscles were more atrophied compared to the right side. Serum creatine kinase levels were elevated, and serologic testings were all negative. Genetic testing using a targeted gene-sequencing panel for neuromuscular disease including myopathy identified no pathogenic variants. Muscle biopsy on the right vastus lateralis showed scattered myofiber necrosis with phagocytosis and an absence of prominent inflammatory cells, consistent with seronegative necrotizing myopathy. Thus, unusual asymmetric muscle weakness and atrophy can be a manifestation of inflammatory myopathy.
免疫介导性坏死性肌病是炎性肌病的一个新亚组，通常起病亚急性，表现为对称性近端肌无力。一名35岁男性，有严重的不对称性髂腰肌萎缩和腰痛，既往有左下肢无力病史。尽管他12年前首次出现左下肢无力，但未得到明确诊断。双侧大腿肌肉磁共振成像显示肌肉水肿和斑片状强化，与右侧相比，左侧臀部和大腿肌肉萎缩更明显。血清肌酸激酶水平升高，血清学检查均为阴性。使用针对包括肌病在内的神经肌肉疾病的靶向基因测序面板进行基因检测，未发现致病变异。右侧股外侧肌肌肉活检显示散在的肌纤维坏死伴吞噬现象，且无明显炎症细胞，符合血清阴性坏死性肌病。因此，不寻常的不对称性肌无力和萎缩可能是炎性肌病的一种表现。
