Prominent inflammatory changes on muscle biopsy in patients with Miyoshi myopathy

Miyoshi myopathy is a rare autosomal recessive distal myopathy characterized by early and prominent involvement of the posterior compartment of the legs. We describe two patients with the clinical diagnosis of Miyoshi myopathy who demonstrated marked inflammatory changes on muscle biopsy of clinically less affected muscles. This report illustrates the importance of recognizing the marked variability in histopathology of Miyoshi myopathy which may include an inflammatory infiltrate on muscle biopsy which mimics the histopathologic picture of an inflammatory myopathy.