TY - JOUR
T1 - Neurodevelopmental evolution of West syndrome
T2 - A 2-year prospective study
AU - Guzzetta, Francesco
AU - Cioni, Giovanni
AU - Mercuri, Eugenio
AU - Fazzi, Elisa
AU - Biagioni, Enrico
AU - Veggiotti, Pierangelo
AU - Bancale, Adina
AU - Baranello, Giovanni
AU - Epifanio, Roberta
AU - Frisone, Maria Flavia
AU - Guzzetta, Andrea
AU - La Torre, Giuseppe
AU - Mannocci, Alice
AU - Randò, Teresa
AU - Ricci, Daniela
AU - Signorini, Sabrina
AU - Tinelli, Francesca
PY - 2008/9
Y1 - 2008/9
N2 - Objective: The aim of this study was to evaluate the epileptic and developmental evolution in infants with West syndrome. Methods: A prospective study of 21 infants was performed, with a follow-up at 2 years. Serial assessment included long-term EEG monitoring, visual and auditory evaluation and assessment of neurodevelopment. Results: Neurosensory and developmental impairments at the spasm onset were transitory in seven cases, including four cryptogenic forms. In all other cases, there was a progressive worsening in neurosensory and developmental impairments. The epileptic evolution was generally better: in 11 of the 16 infants without seizures at outcome, spasms had already disappeared by 2 months after disease onset. Statistic analysis of results showed a correlation between neurosensory impairment and development throughout the whole follow-up. In addition, visual function at T1 resulted significant predictor of developmental outcome. Among the epileptic features, disorganization of slow sleep was an unfavorable prognostic factor. Conclusion: Some forms of West syndrome are confirmed to have a benign evolution: among them there are not only cryptogenic cases but also symptomatic ones without significant neurodevelopmental impairment. Abnormalities of sleep organization, expression of the pervasive epileptic disorder, seem to play a role in determining a developmental deterioration. Neurosensory impairment since the onset of the disease could be a relevant cause of the developmental disorder.
AB - Objective: The aim of this study was to evaluate the epileptic and developmental evolution in infants with West syndrome. Methods: A prospective study of 21 infants was performed, with a follow-up at 2 years. Serial assessment included long-term EEG monitoring, visual and auditory evaluation and assessment of neurodevelopment. Results: Neurosensory and developmental impairments at the spasm onset were transitory in seven cases, including four cryptogenic forms. In all other cases, there was a progressive worsening in neurosensory and developmental impairments. The epileptic evolution was generally better: in 11 of the 16 infants without seizures at outcome, spasms had already disappeared by 2 months after disease onset. Statistic analysis of results showed a correlation between neurosensory impairment and development throughout the whole follow-up. In addition, visual function at T1 resulted significant predictor of developmental outcome. Among the epileptic features, disorganization of slow sleep was an unfavorable prognostic factor. Conclusion: Some forms of West syndrome are confirmed to have a benign evolution: among them there are not only cryptogenic cases but also symptomatic ones without significant neurodevelopmental impairment. Abnormalities of sleep organization, expression of the pervasive epileptic disorder, seem to play a role in determining a developmental deterioration. Neurosensory impairment since the onset of the disease could be a relevant cause of the developmental disorder.
KW - Epilepsy and development
KW - Neurosensory development
KW - West syndrome
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U2 - 10.1016/j.ejpn.2007.10.008
DO - 10.1016/j.ejpn.2007.10.008
M3 - Article
C2 - 18063397
AN - SCOPUS:48549106991
VL - 12
SP - 387
EP - 397
JO - European Journal of Paediatric Neurology
JF - European Journal of Paediatric Neurology
SN - 1090-3798
IS - 5
ER -