Refractory and Super-refractory Status Epilepticus

Context: Refractory status epilepticus (RSE) and super-refractory status epilepticus (SRSE) are neurological emergencies withconsiderable mortality and morbidity. In this paper, we provide an overview of causes, evaluation, treatment, and consequences of RSEand SRSE, reflecting the lack of high-quality evidence to inform therapeutic approach. Sources: This is a narrative review based onpersonal practice and experience. Nevertheless, we searched MEDLINE (using PubMed and OvidSP vendors) and Cochrane centralregister of controlled trials, using appropriate keywords to incorporate recent evidence. Results: Refractory status epilepticus iscommonly defined as an acute convulsive seizure that fails to respond to two or more anti-seizure medications including at least one non-benzodiazepine drug. Super-refractory status epilepticus is a status epilepticus that continues for ≥24 hours despite anesthetic treatment,or recurs on an attempted wean of the anesthetic regimen. Both can occur in patients known to have epilepsy or de novo, with increasingrecognition of autoimmune and genetic causes. Electroencephalography monitoring is essential to monitor treatment response inrefractory/super-refractory status epilepticus, and to diagnose non-convulsive status epilepticus. The mainstay of treatment for thesedisorders includes anesthetic infusions, primarily midazolam, ketamine, and pentobarbital. Dietary, immunological, and surgicaltreatments are viable in selected patients. Management is challenging due to multiple acute complications and long-term adverseconsequences. Conclusions: We have provided a synopsis of best practices for diagnosis and management of refractory/super-refractory status epilepticus and highlighted the lack of sufficient high-quality evidence to drive decision making, ending with a brief forayinto avenues for future research.

Inflicted neuro-trauma in infancy.

Two infants with non-accidental inflicted neuro-trauma are reported. One presented with sudden onset lethargy, respiratory difficulty and unexplained seizures. There were bilateral retinal bleeds and extradural hemmorage. Other was a well thriving child who had 2 seizures and was noted to lack visual fixation. Retinal hemorrhages and chronic subdural and intraparenchymal hemorrhages were subsequently discovered. We highlight the importance of suspecting child abuse in infants with sudden unexplained unresponsiveness, seizures or respiratory difficulty and the unusual occurrence of extradual hemorrhage.

ACTH therapy in refractory generalized epilepsy.

Adrenocorticotropic hormone (ACTH) has a long track record for the treatment of infantile spasms. However, there is paucity of data on the use of ACTH in the treatment of epilepsy beyond infantile spasms. We report the use of ACTH in two children with refractory generalized epilepsy. Both patients responded well. ACTH may be considered as a useful adjunctive therapy in patients with intractable generalized seizures. Side effects and cost however, remain important concerns.