New-Onset Refractory Status Epilepticus: More Investigations, More Questions

Abstract

A 27-year-old previously healthy woman was admitted to the hospital with recurrent seizures. Status epilepticus developed that became refractory to third-line therapy with propofol and barbiturates. The patient had a very extensive diagnostic workup including autoimmune, viral and genetic investigations. A tentative immune therapy was proposed with high doses of steroids and plasma exchanges. Our patient had an inherited heterozygous single nucleotide variant in the sequence c.1280A>G [p.Lys427Arg] of the SMC3 gene that was insufficient to explain the seizures. Surprisingly, IgM antibodies against Japanese encephalitis virus were positive on the serum drawn 11 days after symptom onset, as detected by ELISA and the immunofluorescence antibody (IFA) technique. IgG antibodies were also positive using the IFA technique, but not with ELISA. The same investigations as well as the detection of the viral genome by the q-RT-PCR technique were negative on cerebrospinal fluid. Despite the suspicion of a viral infection, we concluded that our patient had a new-onset refractory status epilepticus of cryptogenic origin. Termination of the status epilepticus was obtained after 47 days, with a possible benefit from the introduction of ketamine.

Intravenous ketamine in status epilepticus

“…There are 26 studies on the use of KET in SE, including 18 single case reports (14 on adults and four on children) and eight case series (five on adults and three on children) available.…”

“…Regarding case reports, seven RSE cases were NCSE or CSE evolving to NCSE, seven were CSE, and one was focal motor SE …”

“…Only two of 25 studies had a prospective study design, and there is not a single controlled study available. A systematic review evaluated efficacy of KET for refractory seizures in the adult and pediatric populations, finding that currently there exists Oxford level 4, grade C evidence to support the use of KET for refractory SE .…”

Status Epilepticus and Beyond: A Clinical Review of Status Epilepticus and an Update on Current Management Strategies in Super-refractory Status Epilepticus

“…Diffusion-weighted imaging lesions may reverse, while persistent changes signify irreversible neuronal damage and gliosis [108]. Ictal and inter-ictal positron emission tomography and single-photon emission computed tomography have been used in several case reports [109-112]. If a surgical lesion is suggested by non-invasive diagnostic studies, subdural EEG grid placement is necessary to further define the target and map eloquent brain regions.…”

“…In adults, focal resection and multiple subpial transections can be considered if a cortical target separated from eloquent brain regions is identified. It is the subject of a number of case reports [109-112], but the exact prevalence of these surgeries is unknown.…”