Researchers at Queen Square, London, review the long-term outcome of therapies in refractory convulsive status epilepticus. Of 596 patients reported (51% of the total of 1168). 201 (35%) died, 79 (13%) had severe neurological deficit, 80 (13%) mild neurological deficit, 22 (4%) with undefined deficit, and 208 (35%) recovered to baseline. The quality of reported outcome data is generally poor, and only broad recommendations for optimal therapy are possible. General anesthesia remains the backbone of therapy, and immediate control is achieved in two-thirds of cases. Agents analyzed include thiopental/pentobarbital, midazolam, propofol, and ketamine, each having advantages and disadvantages. Children are least likely to be treated with propofol because of risk of propofol infusion syndrome, with myocardial failure and high mortality on prolonged infusion. Ketamine is a second-line drug with potential neurotoxic effects. First-line anesthesia therapy should be used with intensive care support and treatment of the underlying cause. Second-line therapies include hypothermia, magnesium and pyridoxine infusions, immunological therapy, ketogenic diet, and neurosurgery. Antiepileptic drug therapy should be used concurrently with anesthesia but outcome data are sparse. Choice of drug regimens include polytherapy with 2 antiepileptic drugs, high-dose, avoid frequent switching, drugs with low interaction potential, predictable kinetics, drugs without renal or hepatic toxicity, and avoidance of GABAergic AEDs. 
COMMENT. The authors comment that the most striking conclusion of their review of literature was the poor quality of outcome data. Only broad recommendations were possible from the analysis of reports. Refractory status epilepticus is heterogeneous and prognosis depends on factors other than treatment, such as age and etiology. General anesthesia is generally effective, and the rate of withdrawal seizures is lower than often quoted. Propofol infusion syndrome is a rare but frequently fatal complication caused by impaired fatty acid oxidation. The hallmarks are metabolic acidosis, lipemia, rhabdomyolysis and myocardial failure. A 10-year-old boy with status epilepticus treated with propofol developed fatal propofol infusion syndrome when a ketogenic diet was initiated.