The early onset epileptic encephalopathy of infantile spasms is frequently associated with acute cognitive regression, long-term learning disability and autistic spectrum disorder. Although there may be a structural basis to the epilepsy, it appears that seizure activity is directly implicated in the process associated with the above disabilities. There are strong indications of the crucial role of temporal lobe dysfunction in children within this and related epileptic regressions including the site of lesions in tuberous sclerosis and the EEG localisation in a later onset epileptic encephalopathy, the Landau-Kleffner-syndrome. Thus the hypothesis for this study was that the temporal lobe is functionally abnormal in children with infantile spasms. This was tested by recording event related potentials, the electrical indicators of the brain's perception and processing of auditory stimuli. The aims of the current study were to describe the normal developmental changes of mismatch-negativity (MMN) and novelty P3 in the first year of life and to identify whether these ERPs are abnormal in children with infantile spasms. The developmental status of infants with infantile spasms was assessed at presentation. The MMN was only shown in a group mean average in control infants. All obligatory and the endogenous P250, P500, Ncl and Nc2 ERP components of the control infants showed age dependent latencies and differed in latency between wakefulness and stage 2 sleep. Using nonparametric calculations infants with infantile spasms had prolonged latencies of the obligatory and endogenous components during both wakefulness and sleep compared to controls. The results of this study support the hypothesis that the auditory processing is interrupted in infants with infantile spasms. As the auditory cortex is very immature during the first year of life it is therefore suggested that infantile spasms may interfere with crucial maturational processes during the first year of life.
| Identifer | oai:union.ndltd.org:bl.uk/oai:ethos.bl.uk:498590 |
| Date | January 2007 |
| Creators | Werner, Klaus-Georg Erich |
| Publisher | University College London (University of London) |
| Source Sets | Ethos UK |
| Detected Language | English |
| Type | Electronic Thesis or Dissertation |
| Source | http://discovery.ucl.ac.uk/1445952/ |
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