Pujar, S;
(2016)
The status epilepticus in childhood outcomes study (STEPSOUT): long-term mortality, neurological, cognitive and neuroimaging outcomes.
Doctoral thesis , UCL (University College London).
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Abstract
Childhood convulsive status epilepticus (CSE) is associated with considerable short-term mortality and morbidity, but data on long-term outcomes are uncertain. In addition, while the current evidence suggests that prolonged febrile seizures (PFS) can result in acute hippocampal injury, whether this evolves into mesial temporal sclerosis (MTS) and cause temporal lobe epilepsy (TLE) in the long-term is uncertain. In the status epilepticus in childhood outcomes study (STEPSOUT), prospective follow-up of a previously identified unique population-based cohort was performed to investigate mortality, morbidity, and structural consequences within 10 years after childhood CSE. Data from STEPSOUT suggest that aetiology is the main determinant of long-term outcomes following CSE in childhood, and CSE characteristics including seizure duration did not affect outcomes. Mortality within 10 years after childhood CSE was 46 times higher than expected in the reference population, but was predominantly seen in children with pre-existing neurological impairments at CSE presentation and the cause of death was usually not seizure related. Similarly, the long-term outcome was generally less favourable for children who had epilepsy and/or neurological impairments at CSE presentation, but was surprisingly good for those who were neurologically normal prior to their initial CSE and survived beyond 30 days after CSE. Children with PFS had a generally favourable outcome, and only a small minority developed TLE and/or MTS. Neuroimaging data demonstrated little evidence of evolving MTS, but suggested that children with PFS may have a higher prevalence of hippocampal developmental malformations. In addition, widespread diffusion tensor imaging changes reflecting disruption of white matter microstructure were apparent in children with PFS. The novel findings in this work are in some instances, contrary to the long-standing perceptions/beliefs about childhood CSE. The implications of these findings for our understanding of the natural history and long-term prognosis after childhood CSE will be further explored in the body of this thesis.
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