Related Experiment Video
Updated: May 4, 2026
06:37
Immunolabelling Myofiber Degeneration in Muscle Biopsies
Published on: December 5, 2019
8.5K
[Necrotizing myopathies: From genetic to acquired forms].
E Salort-Campana1, A M De Paula2, D Figarella-Branger2
1Centre de référence des maladies neuromusculaires et de la SLA, université d'Aix-Marseille, CHU la Timone, avenue Jean-Moulin, 13005 Marseille, France.
La Revue De Medecine Interne
|December 24, 2013
Summary
Necrotizing autoimmune myopathy (NAM) presents with severe muscle damage but lacks inflammation. Diagnosis requires excluding other myopathies and considering clinical, serologic, and pathologic data for this underdiagnosed condition.
Area of Science:
- Neurology
- Immunology
- Pathology
Context:
- Necrotizing myopathies (NM) are characterized by muscle necrosis and regeneration without significant inflammation.
- Necrotizing autoimmune myopathy (NAM) is a distinct subgroup of idiopathic inflammatory myopathies.
- The histological pattern of NM is unspecific, complicating diagnosis.
Purpose:
- To define diagnostic guidelines for necrotizing autoimmune myopathy (NAM).
- To differentiate NAM from other causes of necrotizing myopathies.
- To improve the diagnosis of this rare and potentially underdiagnosed condition.
Summary:
- NAM typically presents as severe proximal myopathy with elevated creatine kinase levels.
- Clinical presentation can mimic muscular dystrophy, necessitating careful evaluation.
- Diagnosis involves excluding other NM causes and integrating clinicoserologic and pathologic findings.
- Associated conditions like anti-SRP, anti-HMGCR antibodies, connective tissue disease, or malignancy are key considerations.
Impact:
- Proposed guidelines aim to enhance clinical diagnosis of NAM.
- Improved diagnostic accuracy can lead to timely and appropriate patient management.
- Further research may identify additional inciting conditions associated with NAM.
Keywords:
Anti-HMGCRAnti-SRPImmune-mediated necrotizing myopathiesMuscular dystrophiesMyopathie nécrosanteMyopathie nécrosante paranéoplasiqueMyopathies associées aux statinesMyopathies nécrosantes auto-immunesMyopathies toxiquesNecrotizing myopathiesStatin-associated autoimmune myopathyToxic myopathy
Related Concept Videos
01:16Necrosis
5.2K
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...
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...
5.2K
01:22Myasthenia Gravis ll: Pathophysiology
53
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...
53
01:16Cellular Injury IV: Necrosis
63
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,...
63
01:28Disorders of the Skeletal Muscle
2.2K
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...
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...
2.2K
01:26Chemical Synapses
9.5K
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...
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...
9.5K
01:26Chemical Synapses
10.9K
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...
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...
10.9K