ACID MALTASE DEFICIENCY: ADULT ONSET

H & E stain	H & E stain
Muscle fibers with vacuoles. The vacuoles are Clear and unrimmed Located in cytoplasmic and subsarcolemmal areas Variably sized: Small and multiple, or Occupy most of fiber area Most prominent in Type I muscle fiber More prominent in some muscles, especially weak ones. Vacuoles contain: Clear or metachromatic purple material

H & E stain

H & E stain

H & E stain

H & E stain

Gomori trichrome stain

Gomori trichrome stain

Gomori trichrome stain

Gomori trichrome stain

Gomori trichrome stain	Plastic sections: Toluidine blue stain

Acid phosphatase positive granules in muscle fibers Present in focal regions in fibers No correlation with large vacuoles
Acid phosphatase stain	Acid phosphatase stain

Vacuoles are more common & larger in type I (pale) muscle fibers

ATPase pH 9.4 stain

Glycogen storage (PAS stain) Diffuse increase in glycogen staining Present in all muscle fibers Vacuoles are not clearly delineated
PAS stain	PAS stain

Glycogen storage, Unusual pattern (PAS stain) Glycogen staining of vacuoles and individual muscle fibers Some increased staining is present in all muscle fibers
PAS stain	PAS stain

Acid maltase deficiency, Adult onset: Fusiform basilar aneurysm