Neuromuscular

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

Also see: Acid maltase deficiency in childhood; Phosphorylase deficiency

Return to Glycogen disorders
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9/14/2006