title: Hemispherotomy in Rasmussen encephalitis: long-term outcome in an Italian series of 16 patients.
abstract: 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.
authors.affiliation: Department of Pediatric Neuroscience, Carlo Besta Neurological Institute, Milan, Italy. Electronic address: Tiziana.Granata@istituto-besta.it.
authors.forename: Tiziana
authors.initials: T
authors.lastname: Granata
authors.affiliation: Department of Pediatric Neuroscience, Carlo Besta Neurological Institute, Milan, Italy; Department of Pediatrics, University of Chieti, Chieti, Italy.
authors.forename: Sara
authors.initials: S
authors.lastname: Matricardi
authors.affiliation: Department of Pediatric Neuroscience, Carlo Besta Neurological Institute, Milan, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Ragona
authors.affiliation: Department of Pediatric Neuroscience, Carlo Besta Neurological Institute, Milan, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Freri
authors.affiliation: Department of Neurophysiology, Carlo Besta Neurological Institute, Milan, Italy.
authors.forename: Marina
authors.initials: M
authors.lastname: Casazza
authors.affiliation: Clinical Epileptology and Experimental Neurophysiology Unit, Carlo Besta Neurological Institute, Milan, Italy.
authors.forename: Flavio
authors.initials: F
authors.lastname: Villani
authors.affiliation: Clinical Epileptology and Experimental Neurophysiology Unit, Carlo Besta Neurological Institute, Milan, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Deleo
authors.affiliation: Department of Neurosurgery, Carlo Besta Neurological Institute, Milan, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Tringali
authors.affiliation: Child Neurology Unit, IRCCS Istituto delle Scienze Neurologiche, Bellaria Hospital, Bologna, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Gobbi
authors.affiliation: Epilepsy Surgery Centre C. Munari, Milan, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Tassi
authors.affiliation: Epilepsy Surgery Centre C. Munari, Milan, Italy.
authors.forename: Giorgio
authors.initials: G
authors.lastname: Lo Russo
authors.affiliation: Neurosurgery Unit, Bambino Gesù Children's Hospital, Rome, Italy.
authors.forename: Carlo Efisio
authors.initials: CE
authors.lastname: Marras
authors.affiliation: Department of Neuroscience, Bambino Gesù Children's Hospital, Rome, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Specchio
authors.affiliation: Department of Neuroscience, Bambino Gesù Children's Hospital, Rome, Italy.
authors.forename: Federico
authors.initials: F
authors.lastname: Vigevano
authors.affiliation: Department of Neuroscience, Bambino Gesù Children's Hospital, Rome, Italy.
authors.forename: Lucia
authors.initials: L
authors.lastname: Fusco
mesh_terms: D000293:Adolescent; D000328:Adult; D001921:Brain / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D002648:Child; D003071:Cognition; D004569:Electroencephalography; D004660:Encephalitis / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D038421:Hemispherectomy; D006801:Humans; D007558:Italy; D008279:Magnetic Resonance Imaging; D008297:Male; D009043:Motor Activity; D012640:Seizures / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
keywords: Epilepsia partialis continua; Epilepsy; Hemispherotomy; Long-term outcomes; Rasmussen encephalitis; Surgery