title: Cognitive competence at the onset of West syndrome: correlation with EEG patterns and visual function.
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.
authors.affiliation: Child Neuropsychiatry, Catholic University, Rome, Italy.
authors.forename: Teresa
authors.initials: T
authors.lastname: Randò
authors.forename: Giovanni
authors.initials: G
authors.lastname: Baranello
authors.forename: Daniela
authors.initials: D
authors.lastname: Ricci
authors.forename: Andrea
authors.initials: A
authors.lastname: Guzzetta
authors.forename: Francesca
authors.initials: F
authors.lastname: Tinelli
authors.forename: Enrico
authors.initials: E
authors.lastname: Biagioni
authors.forename: Giuseppe
authors.initials: G
authors.lastname: La Torre
authors.forename: Roberta
authors.initials: R
authors.lastname: Epifanio
authors.forename: Sabrina
authors.initials: S
authors.lastname: Signorini
authors.forename: Elisa
authors.initials: E
authors.lastname: Fazzi
authors.forename: Eugenio
authors.initials: E
authors.lastname: Mercuri
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cioni
authors.forename: Francesco
authors.initials: F
authors.lastname: Guzzetta
mesh_terms: D002657:Child Development; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology*; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D019957:Motor Skills Disorders; D011594:Psychometrics; D012720:Severity of Illness Index; D013036:Spasms, Infantile / Q000150:complications* / Q000503:physiopathology / Q000523:psychology; D014786:Vision Disorders / Q000209:etiology*