Inclusion Body Myopathy with Dementia & Paget disease of Bone
|
|
|
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 (MSP1)
![](../pics/biopsy/lgd/ibmpaget/vcphelp2.jpg)
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
![](../pics/biopsy/lgd/ibmpaget/vcphelp3.jpg)
H&E stain
|
![](../pics/biopsy/lgd/ibmpaget/vcphelp4.jpg)
H&E stain
|
Grouped atrophy: Myopathic
- Muscle fibers
- Varied size
- Small fibers
- Rounded or Elongated
- Grouped
- May contain vacuoles
- Endomysial connective tissue
|
![](../pics/biopsy/lgd/ibmpaget/lppagetibmmyogrph2.jpg)
H&E stain
|
Myopathy
Fiber sizes: Varied
Small fibers
Shapes: Round or Angular
Cytoplasm: Mildly basophilic
Myonuclei: Large
![](../pics/biopsy/lgd/ibmpaget/vcphemp2.jpg)
H&E stain
|
Fiber Type Features
![](../pics/biopsy/lgd/ibmpaget/vcpatp94lp.jpg)
ATPase pH 9.4 stain
Type 1 muscle fiber predominance
Small & Large fibers are both types
No fiber type grouping.
|
![](../pics/biopsy/lgd/ibmpaget/vcpatp43lp.jpg)
ATPase pH 4.3 stain
|
![](../pics/biopsy/lgd/ibmpaget/vcpatp43mp.jpg)
ATPase pH 4.3 stain
Type 1 muscle fiber predominance
Scattered type 2C (Intermediate-staining) muscle fibers
|
Vacuoles
Rimmed
Shapes: Irregular
Contents: Granular debris
![](../pics/biopsy/lgd/ibmpaget/hppagetibmvacheh.jpg)
H&E stain
|
![](../pics/biopsy/lgd/ibmpaget/vcphehpa.jpg)
H&E stain
|
![](../pics/biopsy/lgd/ibmpaget/vcpvachemp.jpg)
H&E stain
|
![](../pics/biopsy/lgd/ibmpaget/vcpvacgtmp.jpg)
Gomori trichrome stain
|
![](../pics/biopsy/lgd/ibmpaget/vcpvacgthp.jpg)
Gomori trichrome stain
|
Vacuoles
Red-rimmed
Aggregates
Cytoplasmic bodies (Below, Arrow)
|
![](../pics/biopsy/lgd/ibmpaget/vcpgthpa2.jpg)
Gomori trichrome stain
|
VCP Myopathy: Vacuoles
![](../pics/biopsy/lgd/ibmpaget/vcpvacsvvghp3.jpg)
VvG stain
|
Vacuoles
Contain: Fragmented debris
Aggregates
Irregular, dark-stained regions of sarcoplasm
![](../pics/biopsy/lgd/ibmpaget/vcpvacsvvghp4.jpg)
VvG stain
|
Vacuoles
Contain: Fragmented debris
Aggregates
Irregular, dark-stained regions of sarcoplasm
![](../pics/biopsy/lgd/ibmpaget/vcpvacsvvghp5.jpg)
VvG stain
|
VCP Myopathy: Vacuoles
![](../pics/biopsy/lgd/ibmpaget/vcpvacscr2.jpg)
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
![](../pics/biopsy/lgd/ibmpaget/vcpvacscr1a.jpg)
Congo red stain
|
![](../pics/biopsy/lgd/ibmpaget/vcpvacscr3.jpg)
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
![](../pics/biopsy/lgd/ibmpaget/vcpvaccrhp3b.jpg)
Congo red stain
|
![](../pics/biopsy/lgd/ibmpaget/vcpvaccrhphb.jpg)
Congo red stain
|
![](../pics/biopsy/lgd/ibmpaget/vcpnadhlp.jpg)
NADH stain
|
Internal architecture
Small fibers: Stain dark
Vacuoles: Pale staining
![](../pics/biopsy/lgd/ibmpaget/vcpnadh1.jpg)
NADH stain
|
![](../pics/biopsy/lgd/ibmpaget/vcpvacsacp.jpg)
Acid phosphatase stain
|
Acid phosphatase: May stain regions of vacuoles & aggregates
![](../pics/biopsy/lgd/ibmpaget/vcpaggampdamp.jpg)
AMPDA stain
|
Aggregates: AMPDA +
Present in cytoplasm, at edge of vacuoles & within vacuoles
![](../pics/biopsy/lgd/ibmpaget/vcpaggampdahp3.jpg)
AMPDA stain
|
![](../pics/biopsy/lgd/ibmpaget/vcpaggampdahp2.jpg)
AMPDA stain
|
Aggregates: AMPDA +
Present in cytoplasm, at edge of vacuoles & within vacuoles
![](../pics/biopsy/lgd/ibmpaget/vcpaggampdashp.jpg)
AMPDA stain
|
VCP stained inclusions
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
Return to
IBM with Paget's & Dementia
Return to
Distal myopathy
Return to
Muscle biopsies
Return to
Biopsy illustrations
Return to
Neuromuscular home page
Return to
Polyneuropathy Index
4/2/2023