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利比亚班加西特定神经肌肉疾病的描述性流行病学

Descriptive epidemiology of selected neuromuscular disorders in Benghazi, Libya.

作者信息

Radhakrishnan K, el-Mangoush M A, Gerryo S E

出版信息

Acta Neurol Scand. 1987 Feb;75(2):95-100. doi: 10.1111/j.1600-0404.1987.tb07901.x.

Abstract

A 3-year intensive search for selected neuromuscular disorders in Benghazi, yielded 34 patients with Duchenne's muscular dystrophy (25 index cases), 19 with limb-girdle muscular dystrophy (13 index cases), 4 with facioscapulohumeral muscular dystrophy (3 index cases), 3 with opthalmoplegia-plus (all index cases), 13 with polymyositis, 41 with hereditary motor and sensory neuropathy (HMSN) (17 index cases) and 27 with Guillain-Barré Syndrome (GBS). The age-adjusted prevalence rates, on 31 December 1985, per 100,000 population were 6 for Duchenne dystrophy, 3.7 for limb-girdle dystrophy, 0.8 for facioscapulohumeral dystrophy, 0.6 for opthalmoplegia-plus and 7.9 for HMSN (6.4 and 1.5 for Types I and II, respectively). The adjusted average annual incidence of polymyositis was 8.8/mill population; the peak incidence was observed in females in the age-group 20-40. A mean age-adjusted incidence rate for GBS of 1.7/100,000 population per year has been found. The peak age-specific incidence of GBS occurred in the third decade; the sex-dependent difference in the incidence was not significant. No indigenous forms of the disease were encountered and the clinical features differed little from the descriptions in literature. The large family size and high rate of consanguineous marriages contribute to the high frequency of familial disorders, especially those with autosomal recessive inheritance.

摘要

在班加西针对特定神经肌肉疾病进行的为期3年的深入调查中,发现了34例杜氏肌营养不良患者(25例索引病例)、19例肢带型肌营养不良患者(13例索引病例)、4例面肩肱型肌营养不良患者(3例索引病例)、3例眼肌麻痹叠加型患者(均为索引病例)、13例多发性肌炎患者、41例遗传性运动和感觉神经病(HMSN)患者(17例索引病例)以及27例吉兰 - 巴雷综合征(GBS)患者。1985年12月31日,按年龄调整后的每10万人口患病率分别为:杜氏肌营养不良6例、肢带型肌营养不良3.7例、面肩肱型肌营养不良0.8例、眼肌麻痹叠加型0.6例、HMSN 7.9例(I型和II型分别为6.4例和1.5例)。多发性肌炎经调整后的年平均发病率为每百万人口8.8例;发病率高峰出现在20 - 40岁的女性中。已发现GBS的年龄调整后平均发病率为每年每10万人口1.7例。GBS的年龄特异性发病率高峰出现在第三个十年;发病率的性别差异不显著。未发现该疾病的本土形式,其临床特征与文献描述差异不大。家庭规模大以及近亲结婚率高导致家族性疾病的高发病率,尤其是那些常染色体隐性遗传的疾病。

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