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Related Concept Videos

Myasthenia Gravis ll: Pathophysiology01:22

Myasthenia Gravis ll: Pathophysiology

The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...
Necrosis01:16

Necrosis

Necrosis is considered as an “accidental” or unexpected form of cell death that ends in cell lysis. The first noticeable mention of “necrosis” was in 1859 when Rudolf Virchow used this term to describe advanced tissue breakdown in his compilation titled “Cell Pathology”.
Morphological Manifestations of Necrosis
Necrotic cells show different types of morphological appearance depending on the type of tissue and infection. In coagulative necrosis, cells become anucleated and die, but their...
Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

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.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which leads...
Cellular Injury IV: Necrosis01:16

Cellular Injury IV: Necrosis

Necrosis is a form of irreversible cell death caused by severe injury such as ischemia, toxins, or trauma. Unlike programmed cell death, it is an uncontrolled, pathological process that typically provokes inflammation in surrounding tissues.Pathophysiologic ChangesNecrosis begins when cells sustain critical damage, leading to swelling of organelles, particularly mitochondria, and rapid ATP depletion. As energy levels decline, membrane ion pumps fail, leading to calcium influx and eventually,...
Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
Myasthenia Gravis: Diagnostic Tests01:15

Myasthenia Gravis: Diagnostic Tests

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.
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...

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Immunolabelling Myofiber Degeneration in Muscle Biopsies
06:37

Immunolabelling Myofiber Degeneration in Muscle Biopsies

Published on: December 5, 2019

Acquired necrotizing myopathies.

Yves Allenbach1, Olivier Benveniste

  • 1AP-HP, Hôpital Pitié-Salpêtrière, Department of Internal Medecine 1 and Inflammation-Immunopathology-Biotherapy department, Université Pierre et Marie Curie, Paris 6, 75013, Paris, France. yves.allenbach@psl.aphp.fr

Current Opinion in Neurology
|September 3, 2013
PubMed
Summary
This summary is machine-generated.

Necrotizing autoimmune myopathy (NAM) is a treatable condition distinct from muscular dystrophy. Early diagnosis is crucial, as specific autoantibodies can confirm immune-mediated muscle damage.

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Area of Science:

  • Neurology
  • Immunology
  • Pathology

Background:

  • Necrotizing myopathy presents with muscle fiber necrosis and regeneration, often without significant inflammation.
  • This pattern can be seen in muscular dystrophies or acquired myopathies, including those induced by drugs, toxins, or autoimmune processes.

Purpose of the Study:

  • To review acquired myopathies, with a specific focus on Necrotizing Autoimmune Myopathy (NAM).
  • To highlight the discovery and significance of autoantibodies in diagnosing and understanding NAM.

Main Methods:

  • Review of existing literature on necrotizing myopathies.
  • Analysis of clinical presentations, histological findings, and associated autoantibodies in NAM.
  • Correlation of antibody titers with disease activity.

Main Results:

  • Acquired myopathies can have acute or slow progression, potentially mimicking muscular dystrophy.
  • NAM is associated with autoantibodies against signal recognition particle (SRP) or HMG-CoA reductase, particularly in statin-exposed patients.
  • The presence of these antibodies confirms immune-mediated muscle necrosis, with titers correlating to disease activity.

Conclusions:

  • Necrotizing Autoimmune Myopathies are recognized as a distinct, treatable entity.
  • Prompt diagnosis and treatment with immunosuppressants are essential to differentiate NAM from muscular dystrophy.