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A 75-Year-Old Man with Slowly Progressive Leg and Hand Weakness

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A Case-Based Guide to Neuromuscular Pathology

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Abstract

Sporadic inclusion body myositis (sIBM) is a unique type of idiopathic inflammatory myopathies (IIM). It mainly affects men over age 50 years. The disease is characterized by prominent knee extensor and finger flexor weakness, normal or mildly elevated serum creatine kinase level, mixed myopathic and neurogenic motor unit potentials on electromyography, association with an autoantibody against cytosolic 5′-nucleotide 1A (cN1A; NT5C1A), and lack of a favorable response to immunosuppressive therapies. Muscle biopsy plays an essential role in the diagnosis of sIBM. It typically shows the combination of primary endomysial inflammation, rimmed vacuoles, tubulofilamentous inclusions, excessive cytochrome c oxidase (COX)-deficient myofibers, and diffuse upregulation of class I major histocompatibility complex (MHC1). Here we present a case of IBM to illustrate the clinical and pathological features.

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Authors and Affiliations

  1. Departments of Neurology and Pathology, Boston University Medical Center, Boston, MA, USA

    Lan Zhou

  2. Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Chunyu Cai

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  1. Lan Zhou
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  2. Chunyu Cai
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Correspondence to Lan Zhou .

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Editors and Affiliations

  1. Departments of Neurology and Pathology, Boston University Medical Center, Boston, MA, USA

    Lan Zhou

  2. Department of Pathology, Neuropathology Section, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Dennis K. Burns

  3. Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, USA

    Chunyu Cai

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Zhou, L., Cai, C. (2020). A 75-Year-Old Man with Slowly Progressive Leg and Hand Weakness. In: Zhou, L., Burns, D., Cai, C. (eds) A Case-Based Guide to Neuromuscular Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-25682-1_6

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  • DOI: https://doi.org/10.1007/978-3-030-25682-1_6

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-25681-4

  • Online ISBN: 978-3-030-25682-1

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