title: Epilepsy surgery in adult-onset Rasmussen's encephalitis: case series and review of the literature.
abstract: Rasmussen's encephalitis (RE) is a rare immune-mediated condition characterized by drug-resistant focal epilepsy, progressive neurological, and cognitive deficits associated to unilateral hemispheric atrophy. The onset is typically reported in childhood, although adult cases (A-RE) have been described. While surgical strategies in childhood RE are well defined, little is known about usefulness of epilepsy surgery in A-RE patients. We describe clinical features, surgical approach, and outcome of five A-RE patients who underwent epilepsy surgery, and we review the literature with regard to surgical A-RE cases. We retrospectively studied five A-RE patients aged 21-38 years (mean age 22.8 years) who were followed after surgery for a period ranging from to 1 to 6 years. Demographic, electroclinical, and neuroimaging data were systematically reviewed. Four out of five subjects underwent invasive EEG monitoring to define epileptogenic zone. Epilepsy outcome was defined according to Engel's classification. Surgery consisted of frontal corticectomy in three patients, temporal lobectomy in one, combined temporal lobectomy plus insular, and frontobasal corticectomy in the remaining case. No permanent neurological deficits were observed after surgery. At the last follow-up observation, one patient was seizure-free, two subjects experienced rare disabling seizures, another had moderate seizure reduction, and one had no clinical improvement. Our experience, although limited to few cases, suggests that resective surgery in A-RE may play a role in the context of multidisciplinary therapeutical approach of this severe condition. Since the lack of specific data about surgical options, this topic seems to deserve further investigations and more targeted studies.
authors: Epilepsy Unit, Department of Neurology and Psychiatry, "Sapienza" University, Rome, Italy.
authors: Sara
authors: S
authors: Casciato
authors: Carlo
authors: C
authors: Di Bonaventura
authors: Anna Teresa
authors: AT
authors: Giallonardo
authors: Jinane
authors: J
authors: Fattouch
authors: Pier Paolo
authors: PP
authors: Quarato
authors: Addolorata
authors: A
authors: Mascia
authors: Alfredo
authors: A
authors: D'Aniello
authors: Andrea
authors: A
authors: Romigi
authors: Vincenzo
authors: V
authors: Esposito
authors: Giancarlo
authors: G
authors: Di Gennaro
mesh_terms: D000328:Adult; D017668:Age of Onset; D018450:Disease Progression; D000069279:Drug Resistant Epilepsy / Q000601:surgery; D004569:Electroencephalography; D004660:Encephalitis / Q000601:surgery*; D004827:Epilepsy / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000453:epidemiology; D019635:Neurosurgical Procedures / Q000379:methods*; D011183:Postoperative Complications / Q000453:epidemiology; D017397:Prefrontal Cortex / Q000601:surgery; D012189:Retrospective Studies; D013702:Temporal Lobe / Q000601:surgery; D016896:Treatment Outcome; D055815:Young Adult