Neuromuscular

Hereditary Inclusion Body Myopathy: Type 2

Myopathy
  Early
  Late
Vacuoles
Granules
Internal architecture
  Small fibers

Congo red stain

Muscle: General features


H&E stain
Myopathic features
  Muscle fiber size: Varied
  Smaller fibers: May be clustered
  Endomysial connective tissue:
    Not prominently increased

H&E stain
Vacuoles
  In Small, polygonal muscle fibers


NADH stain

Scattered muscle fibers with coarse
  internal architecture or dark staining

NADH stain

ATPase pH 9.4 stain

Type 1 muscle fiber predominance

Vacuolated muscle fibers


H&E stain

H&E stain

H&E stain

Gomori trichrome stain

Gomori trichrome stain

VvG stain

VvG stain

Congo red stain

Congo red stain

Congo red stain


NADH stain


Acid phosphatase stain
HIBM2: Aggregates
  Acid phosphatase positive granules
  Cytoplasmic aggregate

VvG stain

HIBM2 (GNE) Myopathy: Later pathology



H&E stain
Muscle fiber sizes: Varied
Vacuoles: Cytoplasmic with granular, dark-stained contents; Irregular shapes
Endomysial connective tissue: Moderately increased

H&E stain

H&E stain


Gomori trichrome stain
Vacuoles: Internal red-stained material

Gomori trichrome stain

Vacuoles: Granular, dark-stained contents, more at rim

Glycogen
  Some muscle fibers have increased cytoplasmic glycogen
  Some debris near & in vacuoles contains PAS-positive material

Aggregated & Inclusions in muscle fibers
  Some aggregated material may stain for SMI31 (phosphorylated proteins) or p62

Also see: Distal myopathies

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6/7/2016