Creutzfeldt–Jakob disease and inclusion body myositis: Abundant disease‐associated prion protein in muscle

Pathologicalprion protein (PrPSc) is the hallmark of prion diseases affecting primarily the central nervous system. Using immunohistochemistry, paraffin‐embedded tissue blot, and Western blot, we demonstrated abundant PrPSc in the muscle of a patient with sporadic Creutzfeldt–Jakob disease and inclusion body myositis. Extraneural PrPC‐PrPSc conversion in Creutzfeldt–Jakob disease appears to become prominent when PrPC is abundantly available as substrate, as in inclusion body myositis muscle.

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