Early development in Dravet syndrome; visual function impairment precedes cognitive decline

被引：38

作者：

Chieffo, Daniela ^{[1
]}

Ricci, Daniela ^{[1
]}

Baranello, Giovanni ^{[2
]}

Martinelli, Diego ^{[1
]}

Veredice, Chiara ^{[1
]}

Lettori, Donatella ^{[1
]}

Battaglia, Domenica ^{[1
]}

Dravet, Charlotte ^{[1
]}

Mercuri, Eugenio ^{[1
]}

Guzzetta, Francesco ^{[1
]}

机构：

[1] Catholic Univ, Rome, Italy

[2] Ist Besta, Milan, Italy

来源：

EPILEPSY RESEARCH | 2011年 / 93卷 / 01期

关键词：

Dravet syndrome; Early development; Visual function; SEVERE MYOCLONIC EPILEPSY; WEST-SYNDROME; INFANTS; ACUITY; ATAXIA; TERM;

D O I：

10.1016/j.eplepsyres.2010.10.015

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Aim of the study was to describe prospectively the early neuropsychological evolution including the first pre-cognitive stages of the Severe Myoclonic Epilepsy in Infancy (SMEI) or Dravet syndrome. Five cases, four of whom since before a diagnostic evidence of the Dravet syndrome, were followed up. Full clinical assessment including developmental, visual function and behaviour assessments were serially performed. In four cases, a variable onset age of cognitive decline assessed with developmental scales was preceded some months before by an impairment of visual function; the remaining patient during all the course of follow-up till 51 months of age showed a normal development without visual impairment. A cognitive decline with variable onset was generally confirmed in Dravet syndrome. The previous early impairment of visual function seems to herald the cognitive decline and provides useful prognostic information; furthermore, it possibly suggests some clues for a better understanding of the mechanisms of cognitive deterioration in this syndrome. (C) 2010 Elsevier B.V. All rights reserved.

引用

页码：73 / 79

页数：7

共 23 条

[11] VISUAL INATTENTION IN WEST SYNDROME - A NEUROPSYCHOLOGICAL AND NEUROFUNCTIONAL IMAGING STUDY [J].