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

Tiziana Granata, Sara Matricardi, Francesca Ragona, Elena Freri, Marina Casazza, Flavio Villani, Francesco Deleo, Giovanni Tringali, Giuseppe Gobbi, Laura Tassi, Giorgio Lo Russo, Carlo Efisio Marras, Nicola Specchio, Federico Vigevano, Lucia Fusco

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)1106-1119
Number of pages14
JournalEpilepsy Research
Volume108
Issue number6
DOIs
Publication statusPublished - 2014

Keywords

  • Epilepsia partialis continua
  • Epilepsy
  • Hemispherotomy
  • Long-term outcomes
  • Rasmussen encephalitis
  • Surgery

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology
  • Medicine(all)

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