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Intellectual development before and after the onset of infantile spasms : a controlled prospective longitudinal study in tuberous sclerosis

Humphrey, Ayla and MacLean, Cathy and Ploubidis, George B and Granader, Yael and Clifford, Michelle and Haslop, Maisy and Neville, Brian G R and Yates, John R W and Bolton, Patrick F and , Tuberous Sclerosis 2000 Study Group (2014) Intellectual development before and after the onset of infantile spasms : a controlled prospective longitudinal study in tuberous sclerosis. Epilepsia, 55 (1). pp. 108-16. ISSN 1528-1167. DOI UNSPECIFIED

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OBJECTIVE: Infantile spasms (IS) have long been suspected to be a risk factor for impairment in intellectual development, but there are no controlled, prospective longitudinal data in well-characterized conditions to confirm this suspicion. We tested the hypothesis in a longitudinal study of children with tuberous sclerosis (TS), who have a high risk of developing IS. METHODS: Eleven infants with TS were recruited and studied longitudinally using the Mullen Scales of Early Learning. Seizure histories were assessed using a structured parent interview and by review of medical notes. Intellectual development was examined in relation to the onset and length of exposure to IS and other types of seizures. RESULTS: Six children developed IS and five children developed other types of seizure disorders. Among those that developed IS, estimated mean IQ dropped significantly (nonparametric test for trend p = 0.002) from 92 (prior to onset of spasms) to 73 (after exposure to IS for a month or less) and 62 (after exposure to IS for more than a month). By contrast, there was no significant drop in estimated IQ among the five infants exposed to other types of seizure disorders (nonparametric test for trend p = 0.9). All six children exposed to infantile spasms developed clinically significant intellectual impairment. SIGNIFICANCE: These data provide the first clear evidence of clinically significant, dose dependent, impairment in intellectual development following exposure to infantile spasms. The mechanisms underlying this developmental impairment and methods for preventing it require in depth study.

Item Type: Article
Additional Information: Wiley Periodicals, Inc. © 2014 International League Against Epilepsy.
Controlled Keywords: Age of Onset, Child Development, Child, Preschool, Female, Humans, Infant, Intellectual Disability, Intelligence Tests, Male, Neuropsychological Tests, Prospective Studies, Spasms, Infantile, Tuberous Sclerosis
Depositing User: Atira Pure
Date Deposited: 21 May 2014 11:26
Last Modified: 29 Jan 2015 09:10
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