Skeletal muscle in children with congenital myotonic dystrophy in the first year of life.
Muscle biopsies of 9 children with congenital myotonic dystrophy were taken during the first year of life. All but one biopsy were abnormal showing nonspecific changes like an increased variability of fiber size, a selective atrophy of type I or type II fibers or an increased number of intrafusal muscle fibers. Nevertheless correct diagnosis of the disease based on morphological data only is impossible in some cases. A maturational arrest of the muscle is not a obligate feature of the disease. We conclude that muscle biopsy should be postponed after the first year of life.
Müller-Felber
W
W
Friedrich-Baur-Institut, Department of Neurology and Internal Medicine, University of Munich, Germany.
Schmidt-Achert
M
M
Pongratz
D E
DE
D001706:Biopsy; D006801:Humans; D007223:Infant; D009132:Muscles / Q000473:pathology*; D009223:Myotonic Dystrophy / Q000151:congenital / Q000473:pathology*