Abstracts

Rationale: To report a successful termination of chronic hemispheric ESES during sleep by adding clobazam with rapid escalation of dosages in a child with unilateral polymicrogyria, which failed a combination of levetracetam and topiramate.Methods: Single-case report, including history, physical findings, magnetic brain imaging and prolonged video-EEG monitoring sleep and awake-state electroencephalographic findings. Electrophysiological and short term 3 months clinical outcomes following addition of clobazam as adjuvant new anticonvulsant. This is an eleven year old, left-handed boy referred to the Epilepsy Monitoring Unit (EMU) with recurrent convulsions since the age of 18 months for presurgical evaluation. Semiology of present seizures start as tingling sensations around the mouth or tongue, with associated lip smacking, followed by turning to the right side before generalization. Shorter seizures manifests as clenching, drooling and unresponsiveness for 1-3 minutes. Seizures occur monthly. He was onlevetiracetam and topiramate. His general cognitive function was spared with mild behavioural disturbance in the form of hyperactivity and poor memory. His perinatal history was unremarkable.He absent right kidney.He has no distinct dysmorphic features and no focal signs of neurological deficits Psychometric assessment showed an IQ total score of 87% His sleep-state EEG study done 3 yr ago on referral report showed continuous spike and waves discharges. Patient was admitted to the EMU and sleep-EEG recording revealed runs of sharp waves in awake state and ESES during sleep, maximum at C3-P3/ T5 ( top EEG image). Posterior dominant rhythm was normal. Clobazam was started at a dose of 10 mg at bedtime (0.3mg/kg/day), titrated to 20mg on the following night as a single dose then maintained on 20 mg at night and 10 mg at morning on the 3rd day (1mg/kg/day).Topiramate weaning was started.Results: On the 2nd night of clobazam initiation sleep EEG showed termination of ESES with marked reduction of epileptiform discharges (lower EEG image).MRi brain showed unilateral polymicrogyria ( MRI images ). He has behavioral improvement no recurrence of seizures at 3months follow up and routine EEG study showed no discharges during awake and drowsinessConclusions: Rapidly escalated Clobazam dosages is safe and successfully terminate chronic ESES secondary to polymyocrogyria which failed to respond to other AEDs.This might subserve further prospective studies considering clobazam early in the newly diagnosed epilepsy/ESES in polymicrogyria.