Palliative epilepsy surgery in Aicardi syndrome: a case series and review of literature.
OBJECTIVE
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.
Neural Engineering Laboratory, Mayo Clinic, 200 First Street SW, Rochester, MN, 55902, USA, aimenk127@gmail.com.
Aimen S
AS
Kasasbeh
Christina A
CA
Gurnett
Matthew D
MD
Smyth
D061085:Agenesis of Corpus Callosum / Q000473:pathology; D058540:Aicardi Syndrome / Q000150:complications / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D003337:Corpus Callosum / Q000601:surgery; D004351:Drug Resistance; D004569:Electroencephalography; D004827:Epilepsy / Q000209:etiology / Q000601:surgery*; D005123:Eye / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D009460:Neurologic Examination; D019635:Neurosurgical Procedures / Q000379:methods*; D009900:Optic Nerve / Q000002:abnormalities; D010166:Palliative Care / Q000379:methods*; D011788:Quality of Life; D012640:Seizures / Q000209:etiology / Q000601:surgery; D013036:Spasms, Infantile / Q000209:etiology; D016896:Treatment Outcome; D055536:Vagus Nerve Stimulation