TY - JOUR
T1 - Hemispherotomy in Rasmussen encephalitis
T2 - Long-term outcome in an Italian series of 16 patients
AU - Granata, Tiziana
AU - Matricardi, Sara
AU - Ragona, Francesca
AU - Freri, Elena
AU - Casazza, Marina
AU - Villani, Flavio
AU - Deleo, Francesco
AU - Tringali, Giovanni
AU - Gobbi, Giuseppe
AU - Tassi, Laura
AU - Lo Russo, Giorgio
AU - Marras, Carlo Efisio
AU - Specchio, Nicola
AU - Vigevano, Federico
AU - Fusco, Lucia
PY - 2014
Y1 - 2014
N2 - Surgical 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.
AB - Surgical 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.
KW - Epilepsia partialis continua
KW - Epilepsy
KW - Hemispherotomy
KW - Long-term outcomes
KW - Rasmussen encephalitis
KW - Surgery
UR - http://www.scopus.com/inward/record.url?scp=84902256865&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84902256865&partnerID=8YFLogxK
U2 - 10.1016/j.eplepsyres.2014.03.018
DO - 10.1016/j.eplepsyres.2014.03.018
M3 - Article
C2 - 24815913
AN - SCOPUS:84902256865
SN - 0920-1211
VL - 108
SP - 1106
EP - 1119
JO - Epilepsy Research
JF - Epilepsy Research
IS - 6
ER -