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