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Paraneoplastic necrotizing myopathy: clinical and pathological features

M I Levin1, T Mozaffar, M T Al-Lozi

  • 1Department of Neurology, Washington University School of Medicine, St. Louis, MO 63110, USA.

Neurology
|April 1, 1998
PubMed
Summary

Paraneoplastic necrotizing myopathy presents as rapid, severe muscle weakness. Early cancer evaluation is crucial for diagnosis and treatment of this rare condition.

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Area of Science:

  • Neurology
  • Oncology
  • Pathology

Background:

  • Paraneoplastic syndromes are common neurological complications of cancer.
  • Necrotizing myopathy is a rare manifestation, with limited case reports available.
  • This study aims to detail the clinical and pathological characteristics of paraneoplastic necrotizing myopathy.

Observation:

  • Four patients with paraneoplastic necrotizing myopathy were identified over a 10-year period.
  • Patients presented with subacute, symmetric, proximal muscle weakness.
  • Associated cancers included gastrointestinal adenocarcinoma, transitional cell carcinoma, prostatic carcinoma, and non-small cell lung carcinoma.

Findings:

  • Muscle biopsies revealed significant fiber necrosis (8-100%) and intense alkaline phosphatase staining in connective tissue.

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  • Little to no inflammation was observed in muscle tissue.
  • Two patients experienced improvement with corticosteroids and tumor resection, while two passed away.
  • Implications:

    • Paraneoplastic necrotizing myopathy is a severe condition causing rapid, disabling weakness.
    • Distinctive muscle pathology aids in diagnosis.
    • Prompt cancer screening is essential for patients presenting with these clinical and pathological features.