Neuromuscular

Inclusion Body Myopathy with Dementia & Paget disease of Bone

Patient pictures
  Distal weakness
  Scapular winging
Muscle pathology



      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
Grouped atrophy: Myopathic
  • Muscle fibers
    • Varied size
    • Small fibers
      • Rounded or Elongated
      • Grouped
  • Endomysial connective tissue
    • Mild increase

Fiber Type Features

ATPase pH 9.4 stain

Type 1 muscle fiber predominance
Small & Large fibers are both types
No fiber type grouping.


ATPase pH 4.3 stain

Type 1 muscle fiber predominance
Scattered type 2C (Intermediate-staining) muscle fibers

Vacuoles
  Rimmed
  Shapes: Irregular
  Contents: Granular




H&E stain




Gomori trichrome stain
Red-rimmed vacuoles and occasional Aggregates (Cytoplasmic bodies, Below) on Gomori trichrome stain


Vacuoles with Granular, Basophilic Debris
   
Congo red stain

VCP stained inclusions


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


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10/7/2013