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Hereditary Inclusion Body Myopathy: Type 2

Myopathy   Early   Late Vacuoles Granules Internal architecture   Small fibers

Congo red stain

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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

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NADH stain Scattered muscle fibers with coarse   internal architecture or dark staining

NADH stain

ATPase pH 9.4 stain Type 1 muscle fiber predominance

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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 Aggregates & Inclusions in muscle fibers   Some aggregated material may stain for SMI31 (phosphorylated proteins) or p62 Also see: Distal myopathies Return to Neuromuscular Home Page Return to HIBM 2 4/13/2020