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Cognitive competence at the onset of West syndrome: correlation with EEG patterns and visual function

Published online by Cambridge University Press:  17 October 2005

Teresa Randò ,
Giovanni Baranello ,
Daniela Ricci ,
Andrea Guzzetta ,
Francesca Tinelli ,
Enrico Biagioni ,
Giuseppe La Torre ,
Roberta Epifanio ,
Sabrina Signorini  and
Elisa Fazzi
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Teresa Randò
Affiliation:
Child Neuropsychiatry, Catholic University, Rome, Italy.
Giovanni Baranello
Affiliation:
Child Neuropsychiatry, Catholic University, Rome, Italy.
Daniela Ricci
Affiliation:
Child Neuropsychiatry, Catholic University, Rome, Italy.
Andrea Guzzetta
Affiliation:
IRCCS Stella Maris and Child Neuropsychiatry, University of Pisa, Pisa, Italy.
Francesca Tinelli
Affiliation:
IRCCS Stella Maris and Child Neuropsychiatry, University of Pisa, Pisa, Italy.
Enrico Biagioni
Affiliation:
IRCCS Stella Maris and Child Neuropsychiatry, University of Pisa, Pisa, Italy.
Giuseppe La Torre
Affiliation:
Institute of Hygiene, Catholic University, Rome, Italy.
Roberta Epifanio
Affiliation:
IRCCS Foundation Mondino and Child Neuropsychiatry, University of Pavia, Pavia, Italy.
Sabrina Signorini
Affiliation:
IRCCS Foundation Mondino and Child Neuropsychiatry, University of Pavia, Pavia, Italy.
Elisa Fazzi
Affiliation:
IRCCS Foundation Mondino and Child Neuropsychiatry, University of Pavia, Pavia, Italy.
Eugenio Mercuri
Affiliation:
Child Neuropsychiatry, Catholic University, Rome, Italy.
Giovanni Cioni
Affiliation:
IRCCS Stella Maris and Child Neuropsychiatry, University of Pisa, Pisa, Italy.
Francesco Guzzetta
Affiliation:
Child Neuropsychiatry, Catholic University, Rome, Italy.
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Abstract

The aim of this study was to evaluate cognitive development at the onset of West syndrome (WS) with regard to electroencephalogram (EEG) patterns and visual function. Twenty-five patients (14 males, 11 females) at the onset of spasms (T0) in WS and 2 months later (T1) underwent a full clinical evaluation, including neuroimaging, cognitive assessment, video-EEG, and visual function. Mean age of the patients at spasm onset was 5.9 months (SD 2.5; range 2 to 13mo). Cognitive development, assessed with Griffiths Mental Development Scales (GMDS), was generally impaired at T0 and was significantly related to visual function (p<0.001) at both T0 and T1. In general, there was a specific major impairment in the eye–hand coordination scale of the GMDS which tended to disappear after 2 months in less severe cases. At the onset of spasms, sleep EEG organization seemed to be better related to cognitive abilities than awake hypsarrhythmia. These results support a close link between visual function and cognitive competence in WS and provide additional information to improve the understanding of possible mechanisms underlying cognitive impairment.

Type
Original Articles
Information
Developmental Medicine and Child Neurology , Volume 47 , Issue 11 , November 2005 , pp. 760 - 765
Copyright
2005 Mac Keith Press

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