%0 Case Reports
%T Bilateral Rasmussen Encephalitis: Good Outcome Following Hemispherotomy.
%A Trapp N
%A Co DO
%A Rebsamen S
%A Ikonomidou C
%A Ahmed R
%A Knox A
%J Pediatr Neurol
%V 151
%N 0
%D 2024 Feb 20
%M 38041904
%F 4.21
%R 10.1016/j.pediatrneurol.2023.10.011
%X <B>BACKGROUND: </B>Bilateral Rasmussen encephalitis is a rare variant of a debilitating, typically unihemispheric disease with limited treatment options. Few cases with bilateral histopathology have been reported, all with poor seizure control following surgery. Here we report a favorable outcome following hemispherotomy in a four-year-old male with biopsy-confirmed bilateral disease.<BR><B>METHODS: </B>The patient presented with right hemispheric focal seizures with behavioral arrest and over a year progressed to left lower extremity clonic seizures, epilepsia partialis continua, and loss of ambulation, with transient response to steroids and tacrolimus. Histopathology confirmed bilateral disease. The patient developed super-refractory status epilepticus and underwent right functional hemispherotomy 4.5 years after initial presentation. In a 2.5-year follow-up period, an Engel 1D outcome classification was observed with substantially improved quality of life.<BR><B>CONCLUSIONS: </B>Previous reports of bilateral Rasmussen encephalitis describe universally poor outcomes, and hemispherotomy is often considered contraindicated. However, hemispherotomy in a patient with bilateral Rasmussen encephalitis may have a good outcome if seizures are unihemispheric.