抄録
From the histological studies of 36 quadriceps femoris muscle specimens obtained by biopsy from patients with congenital muscular dystrophy, the following characteristic findings were observed; prominent perimysial and endomysial connective tissue infiltration from the eary stage, a little variation in size of the muscle fibers, and rare occurrence of phagocytosis, vacuolar degeneration and hypertrophic fibers.
Histochemical examination showed remarkable deformities of end-plates but other findings were rather similar with those of Duchenne type PMD.
By electron microscopic examination, focal destruction of sarcolemma by fusion with collagen tissue, autolytic body and dislocation of satellite cells were observed as characteristic findings. Peripheral nerves in biopsied muscle specimen were consisted of thin fibers smaller than 6μ in diameter.
