Inclusion body myositis, muscle blood vessel and cardiac amyloidosis, and transthyretin Val122Ile allele

Valerie Askanas, W. King Engel, Renate B. Alvarez, Blas Frangione, Jorge Ghiso, Ruben Vidal

Research output: Contribution to journalArticle

25 Scopus citations


Typical of sporadic inclusion body myositis muscle biopsies are vacuolated muscle fibers containing intracellular amyloid deposits and accumulations of 'Alzheimer-characteristic' proteins. There is no muscle blood vessel or cardiac amyloidosis. We report on a 70-year-old African- American man homozygous for the transthyretin Val122Ile allele who has both sporadic inclusion body myositis and cardiac amyloidosis. His unique pathological features included transthyretin immunoreactivity in prominent muscle blood vessel amyloid and congophilic amyloid deposits within vacuolated muscle fibers.

Original languageEnglish (US)
Pages (from-to)544-549
Number of pages6
JournalAnnals of Neurology
Issue number4
StatePublished - Apr 19 2000


ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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