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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: 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...
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...

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Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models
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Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models

Published on: May 17, 2024

Paraneoplastic myelopathy.

Eoin P Flanagan1, B Mark Keegan

  • 1Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA. flanagan.eoin@mayo.edu

Neurologic Clinics
|November 29, 2012
PubMed
Summary
This summary is machine-generated.

Paraneoplastic myelopathy, a rare cancer complication, presents with specific spinal cord MRI findings and is diagnosed via neural autoantibodies. Early cancer treatment and immunotherapy are key, though outcomes remain challenging.

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Genetic Profiling and Genome-Scale Dropout Screening to Identify Therapeutic Targets in Mouse Models of Malignant Peripheral Nerve Sheath Tumor
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Genetic Profiling and Genome-Scale Dropout Screening to Identify Therapeutic Targets in Mouse Models of Malignant Peripheral Nerve Sheath Tumor

Published on: August 25, 2023

Area of Science:

  • Neurology
  • Oncology
  • Immunology

Background:

  • Paraneoplastic myelopathy is a rare but significant neurological complication associated with underlying cancer.
  • Neurologic symptoms can be the initial presentation, preceding a cancer diagnosis.
  • Understanding this condition is crucial for timely diagnosis and management of both neurological and oncological aspects.

Purpose of the Study:

  • To highlight the diagnostic considerations for paraneoplastic myelopathy.
  • To describe characteristic magnetic resonance imaging (MRI) findings.
  • To outline current management strategies and challenges.

Main Methods:

  • Review of characteristic spinal cord MRI findings, including T2-signal changes and gadolinium enhancement.
  • Emphasis on the role of detecting neural-specific autoantibodies for diagnosis.
  • Discussion of diagnostic approaches integrating neurological and oncological investigations.

Main Results:

  • Characteristic MRI findings include longitudinally extensive, symmetric, tract-specific T2-signal changes, sometimes with enhancement.
  • Detection of neural-specific autoantibodies is vital for confirming the diagnosis and directing the cancer search.
  • Management focuses on identifying and treating the primary cancer, alongside immunotherapy.

Conclusions:

  • Paraneoplastic myelopathy requires careful consideration in patients with myelopathy, as it may indicate an occult malignancy.
  • While immunotherapy combinations are used, evidence-based guidelines are limited, and patient morbidity remains high.
  • Autoimmune myelopathies can also occur independently of cancer or alongside systemic autoimmune disorders.