Which histologic findings are characteristic of congenital muscular dystrophy (CMD) with rigid spine?

Updated: Jul 03, 2019
  • Author: Emad R Noor, MBChB; Chief Editor: Amy Kao, MD  more...
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Answer

Rigid spine with muscular dystrophy (deficiency of selenoprotein N)

  • Myopathic features include small, round muscle fibers, endomysial fibrosis and type 1 fiber predominance or atrophy.

  • Regenerating and degenerating muscle fibers and fiber necrosis are rare. Severe cases may have significant fibrosis but still little or no necrosis.

  • Minicores may be present.


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