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KMID : 0391519930010010186
Journal of the Korean Child Neurology Society
1993 Volume.1 No. 1 p.186 ~ p.192
Three Cases of Central Core Disease.
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Abstract
Central core disease is one of benign childhood myopathies, charactorized by its histological feature. Mitochondrial oxidative enzyme activity is absent in this cental core area. Patients shows motor weakness in early childhood and motor
developmental
delay. The muscle involvement is generalised and symmetrical in the trunk and limbs. The deep tendon reflexes are normal or deminished. Skeletal deformities are not uncommon. Creatine kinase serum levels are normal and the electromyographic
abnormalities are nonspecific. The disease course is benign and the electromyographic abnormalities are nonspecific.
We experienced 3cases of central core disease. The case 1, a 5 year girl, is the first report in our country All of the cases showed motor weakness and motor developmental delay. In the case 1 and 3, skeletal deformities were detected such as hip
joint
contracture, kyphoscoliosis.
They were treated with physial therapy and had operative correction. We report 3 cases of Central core disease with brief revew of the litratures.
Central cors disease with brief revew of the litratures.
KEYWORD
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