Table of Contents

Washington University Experience | MUSCLE | Myopathies with Mitochondrial Pathology | 26A4C (Case 26) muscle_029 - Copy

A subpopulation of fibers shows numerous interfibrillar aggregates of abnormally shaped, large and small mitochondria without more well developed inclusions. (electron micrographs). ---- The complete NP/NM workup of this patient established an inflammatory myopathy with vacuoles, aggregates and mitochondrial pathology (IM-VAMP). This pattern of pathology correlates with a clinical inclusion body myositis syndrome often with patchy, slowly progressive weakness and poor response to immunomodulating agents. Immune stains show features consistent with IM-VAMP including MHC Class I up-regulation by all muscle fibers, cell foci containing CD 4 and CD 8 lymphocytes, and scattered muscle fibers with SMI-31 and LC-3 aggregates. These features overall are consistent with IMVAMP/inclusion body myositis.

---