Fatal reducing body myopathy. ultrastructural and immnunohistochemical observations
BH Kiyomoto, N Murakami, Y Kobayashi, K Nihei… - Journal of the …, 1995 - Elsevier
Two female infants who developed normally during infancy began to have progressive
muscle hypotonia and weakness from 2 years 10 months and 2 years 3 months of ages,
respectively. Both patients had rapidly progressive muscle weakness with death from
respiratory failure at 4 years 11 months and 3 years 9 months, respectively. In addition to
mild inflammation in their muscle biopsies, the most striking finding was the presence of
numerous reducing bodies (RB) in almost all degenerating fibers. By electron microscopy …
muscle hypotonia and weakness from 2 years 10 months and 2 years 3 months of ages,
respectively. Both patients had rapidly progressive muscle weakness with death from
respiratory failure at 4 years 11 months and 3 years 9 months, respectively. In addition to
mild inflammation in their muscle biopsies, the most striking finding was the presence of
numerous reducing bodies (RB) in almost all degenerating fibers. By electron microscopy …