Role of epilepsy surgery in refractory status epilepticus in children.

INTRODUCTION: Status epilepticus (SE) is one of the most common medical emergencies, requiring urgent treatment; nearly 30 % patients develop refractory SE. The role of epilepsy surgery (ES) for refractory SE however remains unclear with empirical evidence limited to single case reports and small case series. The aim of the present study was to determine the clinical presentation, imaging characteristics and outcome of children with refractory SE who underwent emergency ES for refractory SE. MATERIAL AND METHOD: Patients who had SE, failed to respond to escalating medical treatment of SE with/ without pharmacological suppression therapy, and eventually underwent ES were included. RESULTS: There were ten children, 7 boys and 3 girls (range 6 months to 14 years). The age of onset of epilepsy varied from day 2 of life to 12.8 years. The duration of SE prior to surgery was 2-6 days (mean 3.7 days). Four patients had hemimegalencephaly, 3 had focal cortical dysplasia, 2 had Rasmussen's encephalitis, and one had hemispheric porencephalic cyst. The time interval between onset of seizures and ES ranged from 2 months to 8 years (mean 3.1 year). Seven patients underwent hemispherotomy, resection of dysplasia in two and temporo-parieto-occipital disconnection in one. Nine had Engel I outcome and Engel IIIa in one, at follow up of 12-44 months (mean 31 months). CONCLUSION: Emergency ES is an effective treatment option for termination of refractory SE in children with structural pathology, after failure of medical treatment. Patients with refractory SE with focal or hemispheric structural abnormality on MRI, and concordant semiology with/without concordant EEG can be surgical candidates with or without invasive monitoring.

Juvenile Huntington's disease masquerading as progressive myoclonus epilepsy.

Juvenile Huntington's disease (JHD) has an onset before 20 years of age, and is characterized by behavioural issues, epilepsy, rigidity, bradykinesia and dystonia. It contributes to 0.5-5% of all Huntington disease (HD) cases. JHD demonstrates a more rapid progression and is characterised by dystonia, as opposed to the slow progression with predominant chorea seen in adult-onset HD. Seizures are described in 38% of JHD as compared to 2% in the adult onset HD. The different types of seizures reported in JHD are generalized seizures, myoclonus, absence seizures and less commonly tonic and focal seizures with impaired awareness. JHD patients have good seizure control initially and develop drug-resistant epilepsy in the later stages of the disease which is rarely reported. Here, we report the case of a 13 -year-old boy, who initially presented with generalized tonic-clonic seizures followed by myoclonic jerks, with subsequent cognitive decline, ataxia, involuntary movements and drug resistant epilepsy mimicking a progressive myoclonus sepilepsy. His EEG changed from normal background with generalized interictal epileptiform discharges to diffuse slowing with fast activity devoid of epileptiform activity to reflect electroclinical evolution of the disease process.

Efficacy and tolerability of Melatonin vs Triclofos to achieve sleep for pediatric electroencephalography: A single blinded randomized controlled trial.

PURPOSE: To compare Melatonin with Triclofos for efficacy (proportion of successful EEG, need of augmentation, sleep onset latency (SOL), yield of discharges, duration of sleep, presence and grade of artifacts) and tolerability (adverse effect profile). METHODS: A randomized trial was performed (block randomization). All children were advised regarding sleep deprivation, EEG technician administered the drug. EEG was labelled successful if at least 30 min of record could be obtained (sleep with or without awake state). Pediatric neurologist reported the EEG findings-sleep onset latency, epileptiform abnormalities and graded the artifacts (excess beta activity and movement artifacts if present). The parents were interviewed telephonically next day by a pediatric resident for any adverse effects. The parents, pediatric neurologist and pediatric resident were blinded for the drug given. RESULTS: 228 children were randomized (114 each received Melatonin and Triclofos). Both the groups were comparable at baseline for age group and demographic data. The proportion of successful EEG was 89.4% in Melatonin and 91.2% in Triclofos. First dose was effective in 64% in Melatonin and 63.15% in Triclofos group. Augmentation dose was needed in 25.4% in Melatonin and 28% in Triclofos group. Mean total sleep duration was 80 min after Melatonin and 82.39 after Triclofos administration. Adverse effects were observed in 6.14% of Melatonin and 8.65% of Triclofos group. None of the results were statistically significant. CONCLUSION: There was no significant difference between efficacy and tolerability of Melatonin and Triclofos. Melatonin can be safely used to achieve sleep for EEG in children.