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非进行性共济失调综合征的发病机制及预防方面

Pathogenetic and preventive aspects of non-progressive ataxic syndromes.

作者信息

Sanner G

出版信息

Dev Med Child Neurol. 1979 Oct;21(5):663-71. doi: 10.1111/j.1469-8749.1979.tb01684.x.

Abstract

The article reviews the collected knowledge concerning the non-progressive syndromes of ataxic cerebral palsy. Three syndromes are described: (1) simple ataxia (signs of dyssynergia); (2) ataxic diplegia (ataxia with added diplegic spasticity); and (3) dysequilibrium syndrome (defective postural control and equilibrium). The probable aetiological factors in each syndrome are discussed. In simple ataxia prenatal factors dominate; a high proportion of cases have an untraceable aetiology. Genetic factors play a large rôle, and birthweight is usually normal. With ataxic diplegia the aetiology is again most commonly prenatal, but the proportion of cases with an untraceable aetiology is low. Fetal deprivation of supply and perinatal factors, either isolated or in combination, are important. Low birthweight is frequently found. Genetic aetiological factors are the rule in the dysequilibrium syndrome. Possible preventive measures are discussed on the basis of the aetiological pattern. It is concluded that the possibilities of prevention are greatest in ataxic diplegia.

摘要

本文回顾了有关共济失调型脑性瘫痪非进行性综合征的已有知识。文中描述了三种综合征:(1)单纯共济失调(协同失调体征);(2)共济失调性双瘫(共济失调合并双瘫痉挛);(3)平衡失调综合征(姿势控制和平衡缺陷)。讨论了每种综合征可能的病因。在单纯共济失调中,产前因素占主导;很大比例的病例病因不明。遗传因素起很大作用,出生体重通常正常。对于共济失调性双瘫,病因同样最常见于产前,但病因不明的病例比例较低。胎儿供血不足和围产期因素,无论是单独还是合并出现,都很重要。经常发现低出生体重。遗传病因因素在平衡失调综合征中占主导。根据病因模式讨论了可能的预防措施。得出的结论是,共济失调性双瘫的预防可能性最大。

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