title: 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.
authors.affiliation: Department of Intensive Care, Cliniques St-Luc, Université catholique de Louvain, Brussels, Belgium.
authors.forename: Philippe
authors.initials: P
authors.lastname: Dillien
authors.affiliation: Department of Neurology, Cliniques St-Luc, Université catholique de Louvain, Brussels, Belgium; Laboratory of Neurophysiology, Cliniques St-Luc, Université catholique de Louvain, Brussels, Belgium.
authors.forename: Susana
authors.initials: S
authors.lastname: Ferrao Santos
authors.affiliation: Department of Neurology, Cliniques St-Luc, Université catholique de Louvain, Brussels, Belgium; Laboratory of Neurophysiology, Cliniques St-Luc, Université catholique de Louvain, Brussels, Belgium.
authors.forename: Vincent
authors.initials: V
authors.lastname: van Pesch
authors.affiliation: Viral Diseases Unit, Scientific Institute of Public Health (WIV-ISP), Brussels, Belgium.
authors.forename: Vanessa
authors.initials: V
authors.lastname: Suin
authors.affiliation: Viral Diseases Unit, Scientific Institute of Public Health (WIV-ISP), Brussels, Belgium.
authors.forename: Sophie
authors.initials: S
authors.lastname: Lamoral
authors.affiliation: Department of Intensive Care, Cliniques St-Luc, Université catholique de Louvain, Brussels, Belgium.
authors.forename: Philippe
authors.initials: P
authors.lastname: Hantson
keywords: Cryptogenic origin; Japanese encephalitis virus; New-onset refractory status epilepticus; SMC3 gene; Seronegative limbic encephalitis