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Myasthenia gravis is an autoimmune condition affecting neuromuscular transmission, causing generalized weakness in skeletal muscles. Initial diagnoses rely on patients' signs, symptoms, and medical history. The challenge lies in distinguishing myasthenia from other muscular dystrophies. An important diagnostic feature is the significant improvement of symptoms after administering anticholinesterase inhibitors.
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Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
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The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
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Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
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Satellite stem cells or myosatellite cells are quiescent stem cells that Alexander Mauro first identified in 1961. These cells are located between the sarcolemma, the plasma membrane of muscle fibers, and the basal lamina, the connective tissue sheath covering it. These mononucleated cells are activated in response to muscle injury, can transform into myoblasts, and may form or repair muscle fibers. Myosatellite cells can provide additional myonuclei for muscle regeneration or return to a...
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Nondepolarizing neuromuscular blockers prevent the membrane depolarization of muscle cells and inhibit muscle contraction. These are usually administered with anesthetics to achieve complete muscle relaxation. Upon administration, these drugs first block the small, rapidly contracting muscles of the face and hands, followed by the larger muscles of the trunk and the intercostal muscles. The diaphragm is the last muscle to be affected.
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Related Experiment Video

Updated: Jun 30, 2025

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Paraneoplastic myopathies.

Andrew L Mammen1

  • 1Muscle Disease Unit, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, United States; Departments of Neurology and Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, United States.

Handbook of Clinical Neurology
|March 17, 2024
PubMed
Summary
This summary is machine-generated.

Cancer risk varies among idiopathic inflammatory myopathies (IIM). Dermatomyositis and certain immune-mediated necrotizing myopathies show higher cancer risks, unlike antisynthetase syndrome.

Keywords:
Antisynthetase syndromeAutoantibodyCancerDermatomyositisIdiopathic inflammatory myopathyImmune checkpoint inhibitorImmune-mediated necrotizing myopathyInclusion body myositisMyasthenia gravisMyocarditisMyositis

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Area of Science:

  • Neurology
  • Rheumatology
  • Oncology

Background:

  • Idiopathic inflammatory myopathies (IIM) encompass dermatomyositis (DM), antisynthetase syndrome (ASyS), immune-mediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM).
  • The association between IIM and malignancy is a significant clinical concern, impacting diagnosis and management strategies.
  • Understanding cancer risk stratification within IIM subtypes is crucial for patient care.

Approach:

  • This review synthesizes current evidence on the cancer risk associated with different IIM subtypes.
  • It examines specific autoantibodies (e.g., anti-Tif1γ, anti-SRP, anti-HMGCR) and their correlation with malignancy.
  • The role of circulating immune cells (CD57+ CD8+ T cells) in IBM and potential links to leukemia are discussed.

Key Points:

  • Dermatomyositis (DM), particularly with anti-Tif1γ autoantibodies, presents a high risk of coexisting malignancy.
  • Immune-mediated necrotizing myopathy (IMNM) without defined autoantibodies also shows an elevated cancer risk.
  • Inclusion body myositis (IBM) may be associated with large granular lymphocytic leukemia, indicated by increased CD57+ CD8+ T cells.
  • Antisynthetase syndrome (ASyS) and IMNM with anti-SRP or anti-HMGCR autoantibodies do not demonstrate a definitively increased cancer risk.
  • Immune checkpoint inhibitor therapy for cancer can induce myositis (ICI-myositis), potentially with other autoimmune conditions.

Conclusions:

  • Cancer risk is heterogeneous across idiopathic inflammatory myopathies, necessitating subtype-specific evaluation.
  • Certain IIM subtypes and autoantibody profiles serve as important biomarkers for malignancy risk.
  • Further research into the mechanisms linking IIM and cancer, including immune dysregulation, is warranted.