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Palliative epilepsy surgery in Aicardi syndrome: a case series and review of literature

Child's Nervous System volume 30, pages 497–503 (2014)Cite this article

Abstract

Purpose

Aicardi syndrome (AS) is a severe neurodevelopmental disorder characterized by the triad of seizures, agenesis of corpus callosum, and chorioretinal lacunae. Seizures in AS are typically frequent, of various types, and refractory to medical therapy. Optimal treatment of seizures in AS remains undetermined.

Methods

We report a series of four patients with Aicardi syndrome who underwent surgical management of their epilepsy including two with corpus callosotomy (CC) of a partial corpus callosum and three with vagus nerve stimulator implantation.

Results

Seizure outcome was variable and ranged from near complete resolution of seizures to worsening of seizure profile. The most favorable seizure outcome was seen in a patient with partial agenesis of the corpus callosum treated with CC.

Conclusions

Seizure outcome following CC or vagus nerve stimulation in patients with Aicardi syndrome is variable. Although palliative epilepsy surgery may result in improvement in the seizure profile in some patients, studies on larger patient cohorts are needed to identify the precise role that surgery may play in the multidisciplinary approach to controlling seizures in Aicardi syndrome.

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Author information

Affiliations

  1. Neural Engineering Laboratory, Mayo Clinic, 200 First Street SW, Rochester, MN, 55902, USA

    Aimen S. Kasasbeh

  2. Division of Pediatric Neurology, Department of Neurology, Saint Louis Children’s Hospital, Washington University in St. Louis, St. Louis, MO, USA

    Christina A. Gurnett

  3. Department of Neurological Surgery, Saint Louis Children’s Hospital, Washington University in St. Louis, St. Louis, MO, USA

    Matthew D. Smyth

Authors
  1. Aimen S. Kasasbeh
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  2. Christina A. Gurnett
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  3. Matthew D. Smyth
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Correspondence to Aimen S. Kasasbeh.

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Kasasbeh, A.S., Gurnett, C.A. & Smyth, M.D. Palliative epilepsy surgery in Aicardi syndrome: a case series and review of literature. Childs Nerv Syst 30, 497–503 (2014). https://doi.org/10.1007/s00381-013-2259-5

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  • DOI: https://doi.org/10.1007/s00381-013-2259-5

Keywords

  • Aicardi syndrome
  • Epilepsy surgery
  • Corpus callosotomy
  • Vagus nerve stimulation