PMC6230410_01
Trichrome stain showing fibers with multiple vacuoles (arrows). These are commonly seen in acid maltase deficiency and are associated with glycogen accumulation.
CRIHEM2018-9615834.001.jpg
PMC6230410_01_CRIHEM2018-9615834.001.jpg
fig1
A pectoralis muscle biopsy showed increased acid phosphatase in many of the vacuolated fibers suggestive of a vacuolar myopathy, most consistent with acid maltase deficiency (Figures 1 and 2).
Acid phosphatase stain showing increased reactivity (appearing red) in many vacuolated and nonvacuolated fibers. Acid maltase is present in lysosomes, and the increased acid phosphatase reactivity is representative of lysosomal dysfunction.
CRIHEM2018-9615834.002.jpg
PMC6230410_01_CRIHEM2018-9615834.002.jpg
fig2
A pectoralis muscle biopsy showed increased acid phosphatase in many of the vacuolated fibers suggestive of a vacuolar myopathy, most consistent with acid maltase deficiency (Figures 1 and 2).