Neurobehavioral deterioration associated with sleep-augmented epileptiform abnormalities: A steroid responsive state...

Retrospective case record analysis of children with Neurobehavioral Deterioration associated with Sleep-augmented Epileptiform abnormalities (NDSE).

Hospital records of children with NDSE (July, 2015 through December, 2016) were analyzed. Children were categorized as: Encephalopathy with electrical status epilepticus in sleep (ESES) if sleep EEG Spike-wave-Index (SWI) was ≥50% and sleep-induced epileptiform activity (SIEA)-related cognitive dysfunction if SWI ≥25% but <50%. Demography, neurobehavior profile (IQ/SQ and behavior using validated psychometric tools), etiology, investigations and treatment details were documented. Outcome assessment was based on three-month follow-up records.

Eighteen children with NDSE {12 boys; median age at diagnosis: 7.5 years (IQR: 6-10 years); SIEA (7); ESES (11)} were included. Etiology was structural (23%) and presumed genetic (77%). All children received intravenous-methylprednisolone pulse followed by oral steroids for eight weeks. Electroencephalography of children with SIEA was partly organized with median SWI of 40% (IQR 35, 42), with anterior-predominant epileptiform abnormalities and less apparent secondary synchronization. Children with ESES had a disorganized EEG background with median SWI of 80% (IQR 66, 95). Both SIEA and ESES groups had a similar neurobehavior profile. Behavior scores improved in 6/8 children with ESES and 5/7 in SIEA post steroids. In both the groups, median SWI improved (to <5% in SIEA, 45% in ESES). Mild improvement in IQ/SQ was also noted {SIEA [Median (IQR): 3 (1.6, 4.3)]; ESES [Median (IQR): 3.8 (2.8, 7)]}.

The study supports the fact that SWI >50% in the nap EEG is not mandatory for the diagnosis of ESES, thus it should not be a constraint for steroid treatment.