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Related Experiment Videos

Myopathy in primary systemic amyloidosis

M A Gertz1, R A Kyle

  • 1Department of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA.

Journal of Neurology, Neurosurgery, and Psychiatry
|June 1, 1996
PubMed
Summary

Primary systemic amyloidosis with prominent muscle involvement often indicates widespread organ disease and has a poor prognosis. Early diagnosis via monoclonal protein testing is crucial for potential treatment response.

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Area of Science:

  • Neurology
  • Rheumatology
  • Oncology

Background:

  • Primary systemic amyloidosis (PSA) can present with significant muscle involvement.
  • Understanding the natural history of PSA with prominent muscle symptoms is essential for diagnosis and management.

Purpose of the Study:

  • To define the natural history of primary systemic amyloidosis when muscle involvement is a primary presenting symptom.
  • To identify diagnostic clues and prognostic factors in these patients.

Main Methods:

  • Retrospective review of patients with PSA and muscle biopsy-proven amyloid deposits at the Mayo Clinic (1960-1994).
  • Analysis included clinical presentation, diagnostic findings (dysproteinaemia), treatment response, and survival.

Main Results:

  • Twelve patients with muscle amyloidosis were identified; most had widespread visceral involvement (heart, nerve, tongue).
  • All patients had detectable dysproteinaemia; creatine kinase levels were not useful.
  • Median survival was 12 months; three of eight treated with chemotherapy responded.

Conclusions:

  • Monoclonal protein detection is key for diagnosing PSA in patients with muscle weakness.
  • Most patients experience visceral disease, and survival is poor, often due to cardiac failure.
  • Immunoelectrophoresis is recommended for evaluating unexplained myopathy.

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