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Long-term seizure outcome and mobility after surgical treatment for Rasmussen encephalitis in children: A single-center experience.
Nava, Brenda Cristina; Costa, Ursula Thome; Hamad, Ana Paula Andrade; Garcia, Camila Araujo Bernardino; Sakamoto, Americo Ceiki; Aragon, Davi Casale; Machado, Helio Rubens; Santos, Marcelo Volpon.
Afiliación
  • Nava BC; Center for Pediatric Epilepsy Surgery (CIREP), Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
  • Costa UT; Center for Pediatric Epilepsy Surgery (CIREP), Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
  • Hamad APA; Center for Pediatric Epilepsy Surgery (CIREP), Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
  • Garcia CAB; Center for Pediatric Epilepsy Surgery (CIREP), Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
  • Sakamoto AC; Center for Pediatric Epilepsy Surgery (CIREP), Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
  • Aragon DC; Department of Pediatrics, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
  • Machado HR; Center for Pediatric Epilepsy Surgery (CIREP), Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
  • Santos MV; Center for Pediatric Epilepsy Surgery (CIREP), Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
Epileptic Disord ; 25(5): 749-757, 2023 Oct.
Article en En | MEDLINE | ID: mdl-37589547
OBJECTIVE: Rasmussen Encephalitis (RE) is a rare inflammatory neurodegenerative disease associated with refractory seizures, hemiparesis, and cognitive deterioration, due to lateralized cortical atrophy. Hemispheric surgery (hemispherotomy) is the mainstay of treatment, but its unavoidable motor deficits and lack of long-term data regarding seizure outcomes can make patients and families apprehensive to undergo this procedure. The present study aimed at analyzing the results of surgical treatment for RE from a motor and epilepsy standpoint, and mitigate such concerns. METHODS: Clinical and operative data were retrospectively collected from medical records of pharmacoresistant patients treated with functional hemispherectomy at a tertiary reference center for epilepsy surgery, during a 24-year period (1996-2020). Variables such as age of epilepsy onset, seizure semiology, seizure frequency, immunomodulatory therapy, age at surgery, duration of epilepsy, surgical procedures and complications, number of medications used preoperatively and postoperatively were described and statistically analyzed. RESULTS: Forty-three (43) patients were included in this study. Mean age of epilepsy onset was 6.14 years, the average interval between epilepsy onset and hemispherotomy was 2.21 years. and the mean age at surgery was 8.28 years. Thirty patients (69.7%) were Engel I at their last follow-up, of whom 23 (56.4%) were Engel Ia, within a mean follow-up of 11.3 years. Duration of epilepsy, seizure frequency, and age at surgery, among others, did not correlate with seizure outcome, except the use of immunotherapy which led to worse outcomes (p < .05). Also, after surgery, motor functionality was significantly recovered (i.e., most patients returned to their previous status) with time. SIGNIFICANCE: This study tackled some issues regarding the surgical treatment of this disease, particularly showing that hemispherotomy is safe and leads to potentially recoverable disability of motor functions while providing high rates of effective and long-lasting seizure control; therefore, early surgical indication should be warranted once medical refractoriness has been established.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedades Neurodegenerativas / Hemisferectomía / Encefalitis / Epilepsia Límite: Child / Humans Idioma: En Revista: Epileptic Disord Asunto de la revista: CEREBRO / NEUROLOGIA Año: 2023 Tipo del documento: Article País de afiliación: Brasil Pais de publicación: Estados Unidos

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedades Neurodegenerativas / Hemisferectomía / Encefalitis / Epilepsia Límite: Child / Humans Idioma: En Revista: Epileptic Disord Asunto de la revista: CEREBRO / NEUROLOGIA Año: 2023 Tipo del documento: Article País de afiliación: Brasil Pais de publicación: Estados Unidos