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Related Experiment Videos

Myopathy in primary systemic amyloidosis.

M E Roke1, W F Brown, D Boughner

  • 1Department of Clinical Neurological Sciences, University Hospital, London, Ontario, Canada.

The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques
|August 1, 1988
PubMed
Summary

Amyloidosis commonly affects nerves but can also directly weaken skeletal muscles, causing a syndrome of proximal weakness and stiffness. This rare amyloid myopathy often occurs with generalized amyloidosis.

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Area of Science:

  • Neurology
  • Cardiology
  • Pathology

Background:

  • Amyloidosis commonly involves the peripheral nervous system.
  • Direct skeletal muscle infiltration by amyloid is less recognized but significant.

Observation:

  • A case of profound weakness due to amyloid myopathy in a 73-year-old woman with cardiac amyloidosis is presented.
  • Review of 8 similar cases reveals a consistent clinical picture.

Findings:

  • The syndrome is characterized by proximal muscle weakness, stiffness, pseudohypertrophy, and myalgia.
  • Amyloid deposition occurs within the basal lamina of muscle fibers and blood vessels.
  • This myopathy typically develops in patients with established generalized amyloidosis.

Implications:

Related Experiment Videos

  • Highlights the importance of considering amyloid myopathy in generalized amyloidosis.
  • Suggests skeletal muscle involvement is a key feature of systemic amyloidosis.
  • Underscores the need for early diagnosis and management of amyloid myopathy.