Scapular winging in patient with IBM + Paget's

Distal weakness (Severe) in patient with IBM + Paget's Weakness of finger extension: Patchy Foot drop: Myopathic, without anterior leg atrophy

Muscle Pathology: Inclusion Body Myopathy with Dementia & Paget disease

H&E stain

Regional myopathic grouping Groups of small, rounded muscle fibers Mildly increased endomysial connective tissue

H&E stain	Congo red stain
Congo red stain	Congo red stain

Normal & Mutant p97/VCP in vitro: Distribution of VCP & Ubiquitinated proteins

• Normal VCP in cells (Top)
  • VCP staining: Diffusely distributed in cytoplasm
  • Ubiquitinated proteins (FK2 staining): Present in small clusters in cytoplasm
• Mutant VCP in cells (Bottom)
  • VCP staining: Aggregates in cytoplasm
  • Ubiquitinated proteins (FK2 staining): Present in aggregates in cytoplasm
  • VCP & Ubiquitinated proteins: May or not be present in same aggregates