Summary
Unusual inclusions with some of the features of “reducing bodies” were encountered in the skeletal muscle biopsy of a 2.5-year-old boy with childhoodonset acid maltase deficiency. The biopsy revealed a vacuolar myopathy with lysosomal storage of glycogen and eosinophilic refractile inclusions in myofibers, which appeared dark blue with the menadione-nitroblue tetrazolium reaction. The significance of the association of inclusions with reducing properties in the setting of acid maltase deficiency is discussed.
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Jay, V., Christodoulou, J., Mercer-Connolly, A. et al. “Reducing body”-like inclusions in skeletal muscle in childhood-onset acid maltase deficiency. Acta Neuropathol 85, 111–115 (1992). https://doi.org/10.1007/BF00304641
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DOI: https://doi.org/10.1007/BF00304641