Premium
Histopathological Background of Muscle Hypotonia in Children with the Prader‐Willi Syndrome
Author(s) -
Hirayama Yoshito,
Suzuki Haruko,
Ohsawa Makiko
Publication year - 1981
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1981.tb01639.x
Subject(s) - medicine , hypotonia , myofilament , atrophy , pathology , muscle hypotonia , glycogen , anatomy , endocrinology , myocyte
To elucidate the pathogenesis of hypotonia in the Prader‐Willi syndrome, a histological approach towards the five cases was adopted, and traditional histological, histochemical and electron microscopic studies were perfomed. As a result, in light microscopic study type 2 fiber atrophy was found in all the five cases examined, and a combination with type 2 predominance in 3 cases. In addition, localized myopathic change was found in one case and regenerated fiber in another. In electron microscopic study, dilatation of sercoplasmic reticulum, mitochondrial swelling, increased glycogen particles, disappearance of myofilament and partial streaming of Z disc were observed. The presence of these findings led us to propose the hypothesis that there were some disorders in trophic influence on the muscle from the central nervous sytem, mainly in intrauterine life, of subjects with the Prader‐Willi syndrome. (Acta Paediatr Jpn 23(3): 294–300 1981)