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[共济失调毛细血管扩张症患儿的协调障碍]

[Coordination disorders in children with the Louis-Bar syndrome].

作者信息

Aslanov A M

出版信息

Zh Nevropatol Psikhiatr Im S S Korsakova. 1979;79(10):1375-9.

PMID:494913
Abstract

Results of anamnestic and clinico-neurological examinations of 39 children (24 boys and 15 girls aged from 4 to 16 years) with the Louis Barr syndrome are presented. Coordination disorders as the very first symptom were noted in 17 children. In 15 children it was frequent respiratory diseases that were the first manifestations of the syndrome. In these children the coordination disturbances developed later and showed a slower progress. Early development of atactic symptoms and their rapid progress was observed more frequently in boys. Of the coordination disorders prevalent in 22 patients were symptoms of dynamic ataxia more marked in the arms; in 16 patients these symptoms were combined with noticeable disturbances of the statics. The use of both more complicated and simplified modifications of the coordination tests has made it possible to analyze the structure of the atactic syndrome and to estimate the degree of the compensatory potentialities more precisely.

摘要

本文呈现了对39名患有路易-巴尔综合征的儿童(24名男孩和15名女孩,年龄在4至16岁之间)进行回忆性和临床神经学检查的结果。17名儿童最初出现的症状是协调障碍。15名儿童最初表现为频繁的呼吸道疾病。在这些儿童中,协调障碍后来才出现且进展较慢。男孩更常出现共济失调症状的早期发展及其快速进展。在22名患者中普遍存在的协调障碍中,以手臂更明显的动态共济失调症状为主;16名患者这些症状还伴有明显的静态平衡障碍。使用更复杂和简化的协调测试方法,使得分析共济失调综合征的结构并更精确地评估代偿潜力程度成为可能。

相似文献

1
[Coordination disorders in children with the Louis-Bar syndrome].[共济失调毛细血管扩张症患儿的协调障碍]
Zh Nevropatol Psikhiatr Im S S Korsakova. 1979;79(10):1375-9.
2
[Louis-Bar disease. A recent case].
Minerva Pediatr. 1975 May 5;27(16):973-83.
3
[Clinico-genetic diagnosis of ataxia telangiectatica (Louis-Bar syndrome)].
Nervenarzt. 1993 Feb;64(2):140-2.
4
[Ataxia telangiectasia (Louis-Bar syndrome)].[共济失调毛细血管扩张症(路易斯-巴尔综合征)]
Hautarzt. 1979 Oct;30(10):527-31.
5
[On t ataxo-telangiectasia syndrome of Louis-Bar].
Zh Nevropatol Psikhiatr Im S S Korsakova. 1969;69(6):840-3.
6
[Ocular changes in Louis-Bar syndrome (ataxia telangiectasia) in children].
Przegl Lek. 1984;41(5):405-8.
7
Ataxia-telangiectasia. (Clinical and immunological aspects).共济失调毛细血管扩张症。(临床与免疫学方面)
Psychiatr Neurol Med Psychol Beih. 1970;13-14:8-16.
8
[Two cases of ataxia telangiectasia syndrome (Louis-Bar syndrome)].
Zh Nevropatol Psikhiatr Im S S Korsakova. 1966;66(2):168-75.
9
[Eye symptoms in Louis-Bar syndrome].
Ber Zusammenkunft Dtsch Ophthalmol Ges. 1969;69:139-45.
10
[Clinico-immunologic indices in patients with the Louis-Bar syndrome following transplantation of the thymus and sternum].[胸腺和胸骨移植后共济失调毛细血管扩张症患者的临床免疫指标]
Zh Nevropatol Psikhiatr Im S S Korsakova. 1973;73(10):1484-91.

引用本文的文献

1
The natural history of ataxia-telangiectasia (A-T): A systematic review.共济失调毛细血管扩张症(A-T)的自然病史:系统评价。
PLoS One. 2022 Mar 15;17(3):e0264177. doi: 10.1371/journal.pone.0264177. eCollection 2022.