J Korean Neurol Assoc.
1990 Dec;8(2):349-352.
Autosomal Dominant Distal Muscular Dystrophy: Report of A Family
- Affiliations
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- 1Department of Neurology, Yonsei University, Wonju College of Medicine, Korea.
- 2Department of Neurology, Yonsei University, College of Medicine, Korea.
- 3Department of Pathology, College of Medicine, Seoul National Umiversity, Korea.
Abstract
- Ihree cases in a family had progressive muscle weakness and atropy affecting distal hands at first and later involvement of proximal arms and legs. The disorder seemed to be inherited through an autosomal dominant trait with symptom onset in early adulthood, and with slow progression. In one of these patients, the EMG revealed myopathic processes in the muscles of upper extremity and tibialis anterior and gastrocnemius muscles. Biopsy of the left rnedial gastrocnemius muscle showed variation in fiber size, regenerating fibers with vaculoes and mild inflammatory changes. However serum creatine kinase was within normal range. EMG study in others showed similar myopathic processes. The severity and widespreadness of symptoms and EMG abnormalities seemed to be related to the duration of the disease.
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