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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
biochemistry, genetics and molecular biology
Severe childhood muscular dystrophy affecting both sexes and frequent in tunisia
Muscle & Nerve, Volume 6, No. 7, Year 1983
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Description
The authors reported a large study of 93 children presenting a severe form of progressive muscular dystrophy. The first clinical symptoms were noticed between 3 to 12 years. The atrophy affects, predominantly, the girdle and truncal muscles. The hypertrophy of the calves is almost consistent. The progression of the disease is severe, often like that of the Duchenne type. In most of the cases, inability to walk occurs between 10 and 20 years. The serum creatine kinase activity is markedly high in the first stages of the disease. There is a necrotic regenerative pattern at muscle biopsy, associated with a marked type 1 predominance. The disease appears to be inherited as an autosomal recessive trait, with equal distribution among the two sexes. There is a marked variability in the intensity of symptoms and in the severity of the course of the disease from one sibling to another, and from one family to another. This disease is frequent in Tunisia and seems to be related to the high degree of consanguinity in this country. Copyright © 1983 John Wiley & Sons, Inc.
Authors & Co-Authors
Ben-Hamida, Mongi G.M.
Tunisia, Tunis
Institut National de Neurologie Mongi-ben Hamida
Fardeau, Michael
France, Paris
Inserm
Attia, Neïla
Tunisia, Tunis
Institut National de Neurologie Mongi-ben Hamida
Statistics
Citations: 171
Authors: 3
Affiliations: 2
Identifiers
Doi:
10.1002/mus.880060702
ISSN:
0148639X
e-ISSN:
10974598
Research Areas
Disability
Maternal And Child Health
Study Locations
Tunisia