Epilepsy surgery in children with gliomatosis cerebri

Bruno Maton, Trevor Resnick, Prasanna Jayakar, Glenn Morrison, Michael Duchowny

Research output: Contribution to journalArticle

11 Scopus citations

Abstract

Purpose: Gliomatosis cerebri (GC) is a rare neoplastic disorder that may present as intractable epilepsy during early life. We report our experience regarding the evaluation and the surgical treatment of epilepsy in this population. Methods: All children evaluated between 1990 and 2006 for surgery of epilepsy (n = 741) with pathologically proven GC were selected. Results: We identified four male children with age at seizure onset ranging from 4 months to 11 years. Two had hemiparesis and one child with infantile spasms was developmentally delayed. Seizures occurred daily (n = 3) or monthly (n = 1). Ictal semiology was consistent with psychomotor seizures (n = 1), partial motor seizures (n = 2), and asymmetric epileptic spasms (n = 1). Surgery was symptomatic and aimed at debulking and controlling the epilepsy. Procedure was individually tailored based on the presurgical evaluation. Brain MRI revealed widespread hemispheric involvement (n = 3) or infiltration of the temporal lobe and basal ganglia (n = 1). Two patients were initially misdiagnosed as hemispheric cortical dysplasia and hemimegalencephaly. Scalp EEG was nonlocalizing in two cases, showed a right temporal focus in one case, and was not performed in one case. Interictal SPECT in one patient revealed widespread hemispheric hypoperfusion. Three cases were resected under ECoG guidance after a mean delay of 11 months after seizure onset. Following functional hemispherectomy (n = 1) or focal cortical resection (n = 2), all children were alive and seizure free with a mean follow-up of 48 months (2-5 years). No unexpected complication was reported. One nonoperated case was alive but still seizing after 15 months follow-up. Chemotherapy was associated in three cases. Conclusions: GC is a rare cause of medically resistant epilepsy that may present in early life. The lack of a discrete lesion may lead to diagnostic uncertainty, especially in infancy. Epilepsy surgery is an effective therapy that can improve quality of life.

Original languageEnglish (US)
Pages (from-to)1485-1490
Number of pages6
JournalEpilepsia
Volume48
Issue number8
DOIs
StatePublished - Aug 1 2007

Keywords

  • Epilepsy
  • Epilepsy surgery
  • Gliomatosis cerebri
  • Tumor

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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    Maton, B., Resnick, T., Jayakar, P., Morrison, G., & Duchowny, M. (2007). Epilepsy surgery in children with gliomatosis cerebri. Epilepsia, 48(8), 1485-1490. https://doi.org/10.1111/j.1528-1167.2007.01125.x