TY - JOUR T1 - New-Onset Refractory Status Epilepticus: More Investigations, More Questions. JF - Case Rep Neurol Y1 - 2016 A1 - Dillien, Philippe A1 - Susana Ferrao Santos A1 - van Pesch, Vincent A1 - Vanessa Suin A1 - Lamoral, Sophie A1 - Hantson, Philippe KW - Cryptogenic origin KW - Japanese encephalitis virus KW - New-onset refractory status epilepticus KW - Seronegative limbic encephalitis KW - SMC3 gene AB -

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.

VL - 8 CP - 2 U1 - http://www.ncbi.nlm.nih.gov/pubmed/27462243?dopt=Abstract M3 - 10.1159/000447295 ER -