Progressive intellectual impairment in children with Encephalopathy related to Status Epilepticus during slow Sleep

Abstract

We investigated whether Encephalopathy related to Status Epilepticus during slow Sleep (ESES) in childhood was associated with progressive intellectual decline. Participants were identified from the caseload of a single paediatric neurosciences centre and EEG department. A retrospective review of overnight sleep EEG reports ( n =2200) over a five‐year period identified twenty‐two children as having the neurophysiological characteristics of ESES. All had repeat neuropsychological assessment using the WISC‐III (UK) and/or WPPSI‐R (UK). There was a statistically significant reduction in Full‐Scale IQ and Performance IQ across a mean and median time interval of two years. Around a third of the participants showed a clinically significant regression in intellectual functioning evidenced by =>12‐point reduction in IQ. These patients were not distinguishable from the rest of the cohort in terms of clinical history, imaging or duration of ESES. The reduction in IQ reflected reduced processing speed, working memory and overall cognitive efficiency. Children with a history of ESES require close monitoring in order to support educational planning and provide families with accurate information about prognosis.

Continuous Spike–Waves during Slow Sleep Today: An Update

“…The core features of typical ESES, as described by Tassinari et al [12], are (1) electrical-status epilepticus lasting for ≥85% of NREM (slow) sleep, documented by more than two EEG recordings during a period of ≥1 month; (2) different degrees of global cognitive deterioration, with decrease in full-scale IQ and performance IQ [26]; (3) behavioral disorders, including attention deficit, hyperactivity, aggressiveness, difficulty in social interaction, and (more rarely) psychosis (autistic behavior is also possible) [12,[27][28][29]; (4) epileptic seizures, focal or apparently generalized, with a heterogeneous semeiology including absences; epileptic falls; clonic, tonic-clonic, partial motor, and complex partial seizures; and negative myoclonus (see atonic component for example at…”

“…The core features of typical ESES, as described by Tassinari et al [12], are (1) electricalstatus epilepticus lasting for ≥85% of NREM (slow) sleep, documented by more than two EEG recordings during a period of ≥1 month; (2) different degrees of global cognitive deterioration, with decrease in full-scale IQ and performance IQ [26]; (3) behavioral disorders, including attention deficit, hyperactivity, aggressiveness, difficulty in social interaction, and (more rarely) psychosis (autistic behavior is also possible) [12,[27][28][29]; (4) epileptic seizures, focal or apparently generalized, with a heterogeneous semeiology including absences; epileptic falls; clonic, tonic-clonic, partial motor, and complex partial seizures; and negative myoclonus (see atonic component for example at the upper limbs, showed through polygraphic recordings or restricted to lower limbs) can be associated in atypical absences [30][31][32]; it is possible that there are no overt clinical seizures [33], but this is an infrequent occurrence [19,34,35]; and (5) motor signs, including dyspraxia, ataxia, and dystonia. The described clinical EEG characteristics have substantially remained the same over the years [21].…”

Risk factors of electrical status epilepticus during sleep in children with benign childhood epilepsy with centro-temporal spikes

“…It may result in cognitive regression, psychobehavioral abnormalities and neuropsychological function damage in children to some extent, which may affect the long-term prognosis consequently. 4 , 5 Therefore, efforts should be made to suppress the presence of ESES for BECT patients with ESES. However, it is still unclear with respect to the causes of ESES in BECT at present.…”