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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...
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Myocarditis I: Introduction

Myocarditis is inflammation of the myocardium, which is the muscular layer of the heart.EtiologyMyocarditis has a diverse etiology, including a wide range of infectious and non-infectious causes:Infectious CausesViral: Common viruses include Coxsackie A and B, adenovirus, parvovirus B19, enteroviruses, and influenza A.Bacterial: Examples include infections caused by Streptococcus, Staphylococcus, and Mycoplasma species.Rickettsial: Infections like Rocky Mountain spotted fever can result in...
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Myocarditis II: Clinical Features and Diagnostic Tests

Myocarditis is an inflammation of the heart muscle. The symptoms vary widely, encompassing asymptomatic presentations to severe, acute manifestations.Clinical PresentationAsymptomatic cases: In some instances, myocarditis may be asymptomatic, with the infection resolving without intervention. These cases often go undetected unless discovered incidentally through diagnostic imaging or tests conducted for other reasons.General Early Symptoms: Early symptoms of myocarditis are non-specific and can...
Myasthenia Gravis: Overview and Treatment01:20

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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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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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Disorders of the Skeletal Muscle

The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
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Vascular Occlusion Training for Inclusion Body Myositis: A Novel Therapeutic Approach
09:01

Vascular Occlusion Training for Inclusion Body Myositis: A Novel Therapeutic Approach

Published on: June 5, 2010

Inclusion body myositis.

Mazen M Dimachkie1, Richard J Barohn

  • 1Department of Neurology, The University of Kansas Medical Center, Kansas City, Kansas 66160, USA. mdimachkie@kumc.edu

Seminars in Neurology
|November 3, 2012
PubMed
Summary
This summary is machine-generated.

Sporadic inclusion body myositis (IBM) is the most common idiopathic inflammatory myopathy over age 50. While presenting with muscle weakness, IBM is likely degenerative, not inflammatory, with ongoing clinical trials exploring treatments.

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Area of Science:

  • Neurology
  • Rheumatology
  • Rare Diseases

Background:

  • Idiopathic inflammatory myopathies (IIMs) encompass rare disorders like dermatomyositis, polymyositis, necrotizing myopathy, and sporadic inclusion body myositis (IBM).
  • Sporadic IBM is the most prevalent IIM in individuals over 50, characterized by chronic, asymmetric muscle weakness in proximal legs and distal arms.
  • Despite overlapping features with polymyositis, IBM is increasingly recognized as a degenerative condition rather than a primary inflammatory muscle disease.

Purpose of the Study:

  • To provide a comprehensive review of sporadic inclusion body myositis (IBM).
  • To examine the history of clinical trials in IBM, noting their generally negative outcomes.
  • To discuss current and ongoing clinical trials for IBM.

Main Methods:

  • Review of existing literature on idiopathic inflammatory myopathies, focusing on sporadic inclusion body myositis.
  • Analysis of clinical trial data, including past trials with negative results and ongoing investigations.
  • Examination of diagnostic features of IBM, including creatine kinase levels, electromyography, and muscle histopathology.

Main Results:

  • Sporadic inclusion body myositis (IBM) presents with specific patterns of muscle weakness and diagnostic findings.
  • Past clinical trials for IBM have largely yielded negative results, indicating challenges in treatment development.
  • Muscle histopathology in IBM often reveals inflammatory infiltrates and rimmed vacuoles surrounding non-necrotic muscle fibers.

Conclusions:

  • Inclusion body myositis (IBM) is a distinct entity within idiopathic inflammatory myopathies, likely primarily degenerative.
  • The efficacy of past therapeutic interventions for IBM remains unproven.
  • Ongoing clinical trials represent the current frontier for developing effective treatments for sporadic IBM.