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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...
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...
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...
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,...
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...
Chemical Synapses01:26

Chemical Synapses

Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...

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Related Experiment Video

Updated: May 8, 2026

Immunolabelling Myofiber Degeneration in Muscle Biopsies
06:37

Immunolabelling Myofiber Degeneration in Muscle Biopsies

Published on: December 5, 2019

[Necrotizing autoimmune myopathies].

P Petiot1, A Choumert, L Hamelin

  • 1Service de neurologie, centre de référence maladies rares neuromusculaires Rhône-Alpes, groupe hospitalier Nord, 103, grande rue de la Croix-Rousse, 69004 Lyon, France.

Revue Neurologique
|September 4, 2013
PubMed
Summary
This summary is machine-generated.

Necrotizing autoimmune myopathies cause severe muscle damage without typical inflammation. Diagnosis requires muscle biopsy, and treatment involves immunosuppressants with variable responses.

Keywords:
Muscular diseaseMyopathieMyopathie nécrosanteMyopathie paranéoplasiqueNecrotizing myopathyParaneoplasic myopathySRP

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Immunolabelling Myofiber Degeneration in Muscle Biopsies
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Area of Science:

  • Neurology
  • Immunology
  • Pathology

Context:

  • Necrotizing autoimmune myopathies (NAMs) are a subset of inflammatory myopathies.
  • Characterized by significant muscle fiber necrosis without prominent inflammatory infiltrates.
  • Clinical presentation is diverse, often mimicking other idiopathic inflammatory myopathies.

Purpose:

  • To outline the diagnostic features and clinical spectrum of necrotizing autoimmune myopathies.
  • To highlight key pathological findings and associated antibodies.
  • To discuss treatment strategies and response variability.

Summary:

  • NAMs present with severe muscle necrosis, often with rhabdomyolysis and elevated creatine kinase levels.
  • Muscle biopsy reveals necrosis without T-cell invasion, but may show microangiopathy and complement deposition (C5b9).
  • Associated antibodies include anti-SRP and anti-HMGCR (linked to statin-induced NAMs).

Impact:

  • Clarifies the distinct pathological features of NAMs within the inflammatory myopathy spectrum.
  • Aids in accurate diagnosis through characteristic biopsy findings and antibody testing.
  • Informs treatment decisions for this severe autoimmune muscle disease.