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A clinical, epidemiological and genetic study of hereditary motor neuropathies in Benghazi, Libya

✍ Scribed by K. Radhakrishnan; A. K. Thacker; J. C. Maloo


Publisher
Springer
Year
1988
Tongue
English
Weight
239 KB
Volume
235
Category
Article
ISSN
0340-5354

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✦ Synopsis


A 4-year-search for spinal muscular atrophies (hereditary motor neuropathies, HMN) in Benghazi, Libya, yielded a total of 24 patients, among whom 18 were index cases. This group comprised 6 acute infantile, 12 chronic childhood, and 3 each with adult-onset proximal, and distal forms of the disorder. Distal HMN constituted 12.5% of the total cases. The crude average annual incidence of acute infantile HMN was 0.3/100,000 total population and 1/12,500 births in Benghazi. The crude prevalence rates of chronic childhood, adult-onset proximal, and distal types of HMN were 2.3, 0.6, and 0.6/100,000 respectively. The segregation ratios, 0.26 for acute infantile HMN and 0.24 for chronic childhood HMN, suggested autosomal recessive inheritance. The consanguinity rates among parents of cases and the population did not differ significantly.


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Patients affected with hereditary motor sen. sory neuropathy (HMNS) type I were traced through hospital records. Each case was reexamined, a family history was drawn, and EMG examination was performed in those members of the family who could have inherited the trait. In the prevalence year 1987, in