• Title/Summary/Keyword: Inclusion body myositis

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Inclusion Body Myositis : Clinical Features and Electrophysiological Findings (봉입체 근염 : 임상과 전기생리학 소견)

  • Kim, Kwang-Kuk
    • Annals of Clinical Neurophysiology
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    • v.8 no.1
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    • pp.16-22
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    • 2006
  • Sporadic inclusion body myositis (s-IBM) is an aquired slowly progressive inflammatory myopathy with unknown etiology. Although light microscopic abnormalities and characteristic histopathology on muscle biopsy distinguishes from other inflammatory myopathies, vacuolated muscle fibers, intracellular amyloid deposits or tubulofilaments in electromicroscopic findings are not definite in some patients. This review shows the prominently involved muscles in s-IBM and specific or nonspecific electrophysiologic manifestations from reported data for helping the diagnosis of definite-or probable-IBM patients. In lower limbs, the quadriceps is predominantly involved, as is iliopsoas, and tibialis anterior is common. In the upper limbs, the greatest weakness is in forearm finger flexors. Finger extensors, biceps and triceps also are moderately to prominently involved. The majority of patients demonstrate polyphasic MUAPs that are short in duration. An additional striking feature is the concomitant documentation of long-duration, large-amplitude, polyphasic MUAPs. In spite of the frequent mixed myopathic-neurogenic electromyographic findings of IBM, just like that of chronic myositis, asymmetric, slowly progressive weakness of flexor digitorum profundus or quadriceps femoris muscles after age of 50 is very necessary condition for the diagnosis of IBM.

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Inclusion body myositis accompanied with T-cell large granular lymphocyte leukemia

  • Dong-Young Jeong;Seung-Hee Lee;Jungmin So;Ji Yon Kim;Young Chul, Kim;Miyoung Kim;Eun-Ji Choi;Eun-Jae Lee;Hyung Jun Park;Young-Min Lim;Hyunjin Kim
    • Annals of Clinical Neurophysiology
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    • v.25 no.2
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    • pp.106-109
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    • 2023
  • Inclusion body myositis (IBM) is a late-onset myopathy that manifests as distinct muscle weakness in the quadriceps, finger flexors, and ankle dorsiflexors. T-cell large granular lymphocyte (T-LGL) leukemia is a late-onset clonal disorder of CD8+ cytotoxic T-cells that is often accompanied by autoimmune diseases. To date, the association between IBM and T-LGL leukemia has been infrequently reported. Here, we report a case of a patient with T-LGL leukemia who developed IBM, along with in-depth laboratory, electrophysiological, and pathologic findings.

Pathogenesis of Inflammatory Muscle Diseases (염증성 근질환의 발병기전)

  • Lee, Dong Kuck
    • Annals of Clinical Neurophysiology
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    • v.4 no.1
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    • pp.1-6
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    • 2002
  • The inflammatory myopathies are divided into three major and distinct subsets as polymyositis(PM), dermatomyositis(DM), and inclusion body myositis(IBM). This distinction is based on unique clinical, demographic, laboratory, histologic, therapeutic, prognostic, and immunopathologic criteria. Although the causes of PM, DM, and IBM are unknown, autoimmune mechanisms are implicated, as supported by their association with other putative or definite autoimmune diseases or viruses, the evidence for a T cell-mediated myocytotoxicity or complement-mediated microangiopathy, the presence of various autoantibodies and their response to immunotherapies. But in IBM the immune-mediated process is weaker and IBM patients do not readily respond to immunotherapies, there are convincing immunopathological signs to suggest that a definite autoimmune component, similar to that seen in PM, also plays a role in the cause of IBM.

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Rhabdomyolysis Following SARS-CoV-2 Infection in Children: A Case Report and Systematic Review

  • Na-Won Oh;Si-Hwa Gwag;Kyu Sik Cho;Young June Choe
    • Pediatric Infection and Vaccine
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    • v.31 no.1
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    • pp.136-139
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    • 2024
  • Rhabdomyolysis is a syndrome that causes various complications due to the release of substances from muscle cells, often associated with preceding infectious diseases. We report the case of a 7-year-old Korean boy with recent severe acute respiratory syndrome coronavirus 2 infection, presenting with fever, chills, and generalized body aches, diagnosed as rhabdomyolysis. Additionally, we conducted a systematic review with the aim of delineating the disease spectrum, treatment, and outcomes. We identified seven reports that met the inclusion criteria. Among the cases, 5 had fever, with creatine kinase levels ranging from 3,717 and 274,664 IU/L. Two individuals received treatment in intensive care unit, 2 underwent renal replacement therapy, and 1 case has deceased. For children with coronavirus disease 2019 infection and muscle pain, a thorough examination of urine color and an assessment of muscle enzymes through blood tests can help diagnose and treat rhabdomyolysis, a condition that might otherwise be overlooked.