CaSe 1A seven-year-old boy with fever and persisting seizures (generalized tonic-clonic seizures) on multiple anti-epileptic medications {phenytoin (6 mg/kg/day), phenobarbitone (6 mg/kg/day) and valproate (20 mg/kg/day)} was referred to our hospital. At admission, he was in generalized convulsive status epilepticus. Blood investi gations were normal. The dosage of antiepileptic medications was optimized. Cerebrospinal Fluid (CSF) analysis was suggestive of viral meningoencephalitis (lymphocytic leukocytosis, mildly elevated protein, normal glucose with sterile culture) however, specific cultivable viral titers in CSF (Japanese Encephalitis, Herpes Simplex Virus, Dengue, Enterovirus) were negative. Magnetic Resonance Imaging (MRI) brain with contrast showed increased meningeal enhancement and Electroencephalogram (EEG) showed generalized epileptiform activity. He persisted in having seizures on second line Anti-Epileptics Drugs (AEDs) {valproate 40 mg/ kg/day, levetiracetam 60 mg/kg/day} and hence was started on midazolam infusion and mechanically ventilated. Thiopental was added on, following which he remained seizure free for 120 hours with EEG showing burst suppression. Thiopental was gradually tapered and stopped however he had a recurrence of seizures. He was continued on intravenous midazolam, AEDs (Phenytoin 6 mg/kg/day, phenobarbitone 6 mg/kg/day, valproate, levetiracetam, clobazam (10 mg/day) and zonisamide 400 mg/day). He required tracheostomy for prolonged ventilation and had other comorbidities like ventilator-associated pneumonia which were managed accordingly. CSF analysis for Anti-NMDAR and voltage-gated potassium channels were negative. He gradually improved, and seizures subsided with normal EEG.At follow up he had persistent residual neurological deficits (cognition deficits and spasticity) with occasional breakthrough seizures (generalized tonic-clonic seizures). AEDs were gradually tapered and continued on levetiracetam and clobazam. He was under regular follow up for 40 months.
CaSe 2A seven-year-old girl with a history of fever and persisting seizures (generalized tonic-clonic seizures) was admitted. We hereby reported three cases of super refractory seizures as they posed a therapeutic challenge. All three children were aged 6-7 years with prior normal developmental history and no medical illness. Viral meningoencephalitis, fever induced refractory status epilepticus, and auto-immune encephalitis was the probable aetiology in the cases studied. Midazolam, pentobarbital, and ketamine are the most commonly used anaesthetic agents. Phenytoin, phenobarbitone, valproate and levetiracetam are the most commonly used antiepileptic agents. All three cases had residual neurological deficits and morbidities like pneumonia and sepsis. SRSE is associated with high rates of mortality and morbidity necessitating immediate treatment.and CSF analysis including viral titers were normal. MRI brain with contrast showed subtle enhancement along the sulci. EEG showed slow waves with suppressed background [Table...
This open-access article is distributed under Creative Commons licence CC-BY-NC 4.0. Fig. 1. Digital ischaemia of the fingertips following jellyfish sting.
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