Neurodevelopmental evolution of West syndrome: A 2-year prospective study

Autor: Sabrina Signorini, Maria Flavia Frisone, Andrea Guzzetta, Pierangelo Veggiotti, Giovanni Baranello, Elisa Fazzi, E Biagioni, Alice Mannocci, Daniela Ricci, Teresa Randò, Francesco Guzzetta, Eugenio Mercuri, Francesca Tinelli, Giovanni Cioni, Roberta Epifanio, Giuseppe La Torre, A Bancale
Rok vydání: 2008
Předmět:
Male
growth /&/ development/physiopathology
Pediatrics
Developmental Disabilities
Disease
Electroencephalography
Infantile
Spasms
Cohort Studies
Disability Evaluation
Prospective Studies
Child
Prospective cohort study
Neurologic Examination
medicine.diagnostic_test
Brain
West Syndrome
General Medicine
Prognosis
diagnosis/etiology/physiopathology
epilepsy and development
neurosensory development
west syndrome
Child
Preschool
Predictive value of tests
Disease Progression
growth /&/ development/physiopathology
Child
Preschool
Cohort Studies
Developmental Disabilities
diagnosis/etiology/physiopathology
Disability Evaluation
Disease Progression
Electroencephalography
Epilepsy
diagnosis/etiology/physiopathology
Female
Follow-Up Studies
Humans
Infant
Infant
Newborn
Male
Neurologic Examination
Predictive Value of Tests
Prognosis
Prospective Studies
Sleep Disorders
diagnosis/etiology/physiopathology
Spasms
complications/diagnosis/physiopathology
Vision Disorders
Female
Spasms
Infantile
Eeg monitoring
Cohort study
Sleep Wake Disorders
medicine.medical_specialty
Vision Disorders
Predictive Value of Tests
medicine
Humans
Preschool
Psychiatry
Epilepsy
business.industry
Infant
Newborn
Infant
Newborn
medicine.disease
Developmental disorder
Pediatrics
Perinatology and Child Health
complications/diagnosis/physiopathology
Neurology (clinical)
Sleep Disorders
business
Follow-Up Studies
Zdroj: European Journal of Paediatric Neurology. 12:387-397
ISSN: 1090-3798
DOI: 10.1016/j.ejpn.2007.10.008
Popis: 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.
Databáze: OpenAIRE
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