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Inclusion Body Myopathy with Dementia & Paget disease of Bone

Patient pictures   Distal weakness   Scapular winging Muscle pathology

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	Scapular winging in patient with IBM + Paget's

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

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Muscle Pathology: Inclusion Body Myopathy with Dementia & Paget disease (MSP1)

Myopathy Vacuoles Aggregates

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H&E stain

VCP pathology: Features
  Muscle fibers
    Atrophy: Often in clusters
    Hypertrophy
    Nuclei: Internal
    Vacuoles: Irregular shapes; May contain granular debris
    Aggregates
  Endomysial connective tissue: Mildly increased

H&E stain

H&E stain	Grouped atrophy: Myopathic Muscle fibers Varied size Small fibers Rounded or Elongated Grouped May contain vacuoles Endomysial connective tissue Mild increase
	H&E stain

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Myopathy
  Fiber sizes: Varied
  Small fibers
    Shapes: Round or Angular
    Cytoplasm: Mildly basophilic
    Myonuclei: Large

H&E stain

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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

ATPase pH 4.3 stain Type 1 muscle fiber predominance Scattered type 2C (Intermediate-staining) muscle fibers

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Vacuoles   Rimmed   Shapes: Irregular   Contents: Granular debris H&E stain	H&E stain
H&E stain

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Gomori trichrome stain	Gomori trichrome stain
Vacuoles   Red-rimmed Aggregates   Cytoplasmic bodies (Below, Arrow)
Gomori trichrome stain

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VCP Myopathy: Vacuoles

VvG stain

Vacuoles
  Contain: Fragmented debris
Aggregates
  Irregular, dark-stained regions of sarcoplasm

VvG stain

Vacuoles
  Contain: Fragmented debris
Aggregates
  Irregular, dark-stained regions of sarcoplasm

VvG stain

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VCP Myopathy: Vacuoles

Congo red stain

Vacuoles
  Contain: Fragmented debris
  Shapes: Irregular
  Sizes: Varied; Small or Most of fiber area
  Number: One, or several, in individual muscle fibers

Congo red stain

Congo red stain

Vacuoles
  Contain: Fragmented debris
  Shapes: Irregular
  Sizes: Varied; Small or Most of fiber area
  Number: One, or several, in individual muscle fibers
Vacuoles
  Shapes: Irregular
  Contents & Neighboring areas
    Debris: Granular, Basophilic
Myonuclei in vacuolar fibers
  Size: Large
  Mildly pale staining

Congo red stain

Congo red stain

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NADH stain

Internal architecture
  Small fibers: Stain dark
  Vacuoles: Pale staining

NADH stain

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Acid phosphatase stain

Acid phosphatase: May stain regions of vacuoles & aggregates

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AMPDA stain

Aggregates: AMPDA + Present in cytoplasm, at edge of vacuoles & within vacuoles

AMPDA stain

AMPDA stain

Aggregates: AMPDA + Present in cytoplasm, at edge of vacuoles & within vacuoles

AMPDA stain

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VCP stained inclusions

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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

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Return to IBM with Paget's & Dementia
Return to Distal myopathy
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Return to Biopsy illustrations
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4/2/2023