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[一例脑穿通畸形的神经心理学发现]

[Neuropsychological findings in a case of porencephaly].

作者信息

Matarín M M, Arasanz C, Oms B, Olivella J, Junqué C

机构信息

Servicio de Neurología, Hospital de la Cruz Roja, Barcelona, España.

出版信息

Rev Neurol. 1999;29(12):1164-9.

Abstract

INTRODUCTION

At the present time porencephalia refers to the presence of deep, unilateral or bilateral cavities or excavations, frequently communicating with the subarachnoid space or lateral ventricles, which occur following brain destruction during the end of the foetal or beginning of the new-born period. The clinical features are various forms of cerebral paralysis (spastic hemiparesia, diplegia and tetraplegia), mental retardation and epileptic crises. To date there are few studies giving detailed analysis of the cognitive functions of affected persons.

CASE REPORT

We studied the mental and cognitive state of an adult woman with massive porencephalic dilatation of the left lateral ventricle. The patient had a normal verbal intelligence quotient. Cortical function, including language, praxis and gnosis were maintained. She had alterations of memory, particularly verbal, and of frontal function.

CONCLUSIONS

The deficits observed were not lateralized for neocortical functions, but were lateralized for memory. The neuropsychological pattern partly corresponded to subcortical deterioration due to the lesions of the white matter and basal ganglio.

摘要

引言

目前,孔洞脑是指存在深部的单侧或双侧空洞或腔隙,常与蛛网膜下腔或侧脑室相通,这些空洞或腔隙是在胎儿期结束或新生儿期开始时脑破坏后出现的。其临床特征为各种形式的脑瘫(痉挛性偏瘫、双瘫和四肢瘫)、智力发育迟缓及癫痫发作。迄今为止,很少有研究对患者的认知功能进行详细分析。

病例报告

我们研究了一名成年女性的心理和认知状态,该患者左侧侧脑室存在巨大的孔洞脑扩张。患者的言语智商正常。包括语言、运用和认知在内的皮质功能得以保留。她存在记忆改变,尤其是言语记忆,以及额叶功能改变。

结论

观察到的缺陷在新皮质功能方面并非偏侧化,但在记忆方面是偏侧化的。神经心理学模式部分与因白质和基底神经节病变导致的皮质下功能衰退相符。

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