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[共济失调型脑性瘫痪与脑成像]

[Ataxic cerebral palsy and brain imaging].

作者信息

Imamura S, Tachi N, Tsuzuki A, Sasaki K, Hirano S, Tanabe C, Sakuma K

机构信息

Sapporo Municipal Rehabilitation Center for Disabled Children.

出版信息

No To Hattatsu. 1992 Sep;24(5):441-8.

PMID:1389327
Abstract

Five cases diagnosed as having ataxic cerebral palsy were presented with their brain imaging. Case 1, a 3-year-old-girl had been floppy since 7 months of age and began ataxic walk with spastic legs from 18 months of age. MRI revealed generalized atrophy of cerebellum (especially in anterior superior part) and slight atrophy of pons. Her mother also had ataxia with spastic legs of early onset. She and her mother were thought to have an early-onset inherited non-progressive cerebellar ataxia syndrome. Case 2, a 8-year-old-girl had ataxic walk since 17 months of age. MRI revealed cerebellar atrophy especially in anterior superior part. Case 3, a 10-year-old boy was floppy since 4 months of age and suspected as ataxic at 4 years of age. He could walk only with cruches. He had dwarfism and cataracts since 4 years of age. CT and MRI revealed generalized spino-ponto-cerebellar atrophy. Final diagnosis was Marinesco-Sjörgren syndrome. Case 4, a 10-year-old girl had opisthotonus and floppiness since 4 months of age. She could walk only with cruches. CT and MRI revealed generalized spino-ponto-cerebellar atrophy. Case 5, a 8-year-old boy showed head nodding and nystagmus since 4 months of age. He started ataxic gait at 8 years of age. He could vocalize only single sound for speech. MRI revealed cranium bifida and agenesis of anterior medullar velum. Ataxic cerebral palsy is the term often used to describe very different conditions, the clinical picture starts as hypotonia and changes into the ataxic symptoms in a few years.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

本文介绍了5例被诊断为共济失调型脑性瘫痪患者的脑部影像学检查结果。病例1为一名3岁女孩,自7个月大起出现肌张力低下,18个月大时开始出现伴有痉挛性腿部的共济失调步态。MRI显示小脑广泛性萎缩(尤其是前上部)和脑桥轻度萎缩。她的母亲也有早发性共济失调伴痉挛性腿部症状。她和她的母亲被认为患有早发性遗传性非进行性小脑共济失调综合征。病例2为一名8岁女孩,自17个月大起出现共济失调步态。MRI显示小脑萎缩,尤其是前上部。病例3为一名10岁男孩,自4个月大起出现肌张力低下,4岁时被怀疑患有共济失调。他只能借助拐杖行走。自4岁起患有侏儒症和白内障。CT和MRI显示广泛性脊髓-脑桥-小脑萎缩。最终诊断为马里内斯科-施约格伦综合征。病例4为一名10岁女孩,自4个月大起出现角弓反张和肌张力低下。她只能借助拐杖行走。CT和MRI显示广泛性脊髓-脑桥-小脑萎缩。病例5为一名8岁男孩,自4个月大起出现点头和眼球震颤。8岁时开始出现共济失调步态。他只能发出单音。MRI显示颅骨裂和延髓前膜发育不全。共济失调型脑性瘫痪这一术语常被用于描述非常不同的病症,临床表现始于肌张力减退,并在几年内转变为共济失调症状。(摘要截断于250字)

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