Hemispherotomy in Rasmussen encephalitis: Long-term outcome in an Italian series of 16 patients

•Hemispherotomy is treatment of choice in RE but long-term data are not available.•We present long-term outcomes in 16 patients with RE who underwent hemispherotomy.•13 patients were seizure-free; drugs were withdrawn in 10 and reduced in 6.•Early hemispherotomy favors good motor and cognitive outcomes.•Hemispherotomy should be considered early in disease course.

SummarySurgical disconnection of the affected hemisphere is considered the treatment of choice for Rasmussen encephalitis (RE), however few data on long-term outcomes after disconnective surgery are available. We report on long-term seizure, cognitive and motor outcomes after disconnective surgery in 16 (8 M, 8 F) RE patients.Pre- and post-operative evaluations included long-term video-EEG monitoring, MRI, assessment of motor function, and cognitive evaluation. Hemispherotomy, by various techniques was used to obtain functional disconnection of the affected hemisphere.The patients, of median current age 23.5 years, range 12–33, were operated on between 1993 and 2009. Median age at disease onset was 5.8 years (range 3–11.4). Median time from seizure onset to surgery was 3.8 years, range 8 months to 21 years. Post-surgical follow-up was a median of 9.5 years, range 3–20. At surgery all patients were receiving two or more antiepileptic drugs (AEDs). All but three patients were seizure-free at latest follow-up. AEDs had been stopped in ten patients; in the remaining six AEDs were markedly reduced. Postural control improved in all patients. Gain in cognitive functioning was significantly (p = 0.002) related to disease duration.The long-term outcomes, in terms of seizure control, motor improvement, and cognitive improvement provide important support for disconnective surgery as first choice treatment for RE.