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婴儿发作性偏瘫伴全面性癫痫发作伴热性惊厥附加症的神经认知和行为特征。

Neurocognitive and behavioural profile in Panayiotopoulos syndrome.

机构信息

Department of Neurology, Maastricht University Medical Center+, Maastricht, the Netherlands.

Kempenhaeghe Epilepsy Center, Heeze, the Netherlands.

出版信息

Dev Med Child Neurol. 2020 Aug;62(8):985-992. doi: 10.1111/dmcn.14417. Epub 2019 Dec 3.

Abstract

AIM

To determine neurocognitive performance and behavioural problems in children with Panayiotopoulos syndrome.

METHOD

All 18 children (10 females, 8 males; mean age 4y 7mo; SD 1y 10mo) diagnosed with Panayiotopoulos syndrome at the Kempenhaeghe Epilepsy Center in the Netherlands between 2010 and 2017 were analysed retrospectively. All underwent a neuropsychological/behavioural assessment, an academic assessment, and a 24-hour electroencephalogram.

RESULTS

Mean full-scale IQ (93.5; range 76-123; p=0.04) and performance IQ (93.2; range 76-126; p=0.04) were within the normal range, although significantly lower compared to the normative mean. Verbal IQ (96.3; range 76-118) and processing speed (96.1; range 74-114) were not significantly lower. Simple auditory/visual reaction times, visual attention, visual-motor integration, and verbal memory were significantly lower compared to normative values. On average, patients with Panayiotopoulos syndrome were 8 months behind in arithmetic speed and 11 months behind in reading speed for the number of months in school. Behavioural questionnaires revealed significantly higher scores on reported internalizing behavioural problems.

INTERPRETATION

Children with Panayiotopoulos syndrome demonstrated diffuse cognitive dysfunction in full-scale IQ, performance IQ, visual attention, visual-motor integration, and verbal memory. A high incidence of internalizing behavioural problems was reported. This strongly suggests neuropsychological and behavioural comorbidity in children with Panayiotopoulos syndrome.

WHAT THIS PAPER ADDS

Children with Panayiotopoulos syndrome are at risk for cognitive deficits in various cognitive domains. Children with Panayiotopoulos syndrome are also prone to internalizing behavioural problems. Mild-to-severe academic underachievement was present in more than half of the children with Panayiotopoulos syndrome.

摘要

目的

确定 Panayiotopoulos 综合征患儿的神经认知表现和行为问题。

方法

回顾性分析 2010 年至 2017 年期间在荷兰 Kempenhaeghe 癫痫中心诊断为 Panayiotopoulos 综合征的 18 名儿童(10 名女性,8 名男性;平均年龄 4 岁 7 个月;标准差 1 岁 10 个月)。所有儿童均接受神经心理学/行为评估、学业评估和 24 小时脑电图检查。

结果

平均全量表智商(93.5;范围 76-123;p=0.04)和操作智商(93.2;范围 76-126;p=0.04)在正常范围内,但与正常均值相比显著较低。言语智商(96.3;范围 76-118)和加工速度(96.1;范围 74-114)没有明显较低。简单听觉/视觉反应时间、视觉注意力、视觉运动整合和言语记忆明显低于正常值。平均而言,Panayiotopoulos 综合征患儿在算术速度方面落后 8 个月,在阅读速度方面落后 11 个月。行为问卷显示,报告的内化行为问题得分明显较高。

解释

Panayiotopoulos 综合征患儿在全量表智商、操作智商、视觉注意力、视觉运动整合和言语记忆方面表现出弥散性认知功能障碍。报告内化行为问题的发生率较高。这强烈表明 Panayiotopoulos 综合征患儿存在神经认知和行为共病。

本文添加内容

Panayiotopoulos 综合征患儿存在各种认知领域认知功能缺陷的风险。Panayiotopoulos 综合征患儿也易发生内化行为问题。超过一半的 Panayiotopoulos 综合征患儿存在轻度至重度学业成绩不良。

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