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.
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.
Authors: Hussan S Mohammed; Christian B Kaufman; David D Limbrick; Karen Steger-May; Robert L Grubb; Steven M Rothman; Judith L Z Weisenberg; Rebecca Munro; Matthew D Smyth Journal: Epilepsia Date: 2012-02-07 Impact factor: 5.864
Authors: C M Uchiyama; C M Carey; W B Cherny; D L Brockmeyer; L D Falkner; M L Walker; R S Boyer Journal: Pediatr Neurosurg Date: 1997-08 Impact factor: 1.162
Authors: Brian J Dlouhy; Brandon Miller; Anna Jeong; Mary E Bertrand; David D Limbrick; Matthew D Smyth Journal: Childs Nerv Syst Date: 2016-07-27 Impact factor: 1.475