Palliative epilepsy surgery in Aicardi syndrome

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

Authors: Aimen S. Kasasbeh & Christina A. Gurnett & Matthew D. Smyth


to analyze the anticonvulsive effect of palliative surgery (such as corpus callosotomy-CC and vagal nerve stimulation-VNS) in patients with Aicardi Syndrome (AS) and refractory epilepsy.


case study of 4 patients with AS and refractory epilepsy. One patient was treated with CC (1); one patient with VNS and thereafter CC (2) and two patients with VNS only (3,4).


In patient 1 CC was performed at the age of 7 years. One month after CC atonic seizures had disappeared and she showed improved receptive language. At 20 months there was a dramatic improvement which continued thereafter. Absence and drop attacks ceased and cognitive and social development kept going. There was > 90% reduction of other seizure types. In patient 2 VNS was implanted elsewhere (no age at implantation given) and turned off again on request of the parents because of worsening of seizures. At the age of 7 years CC was performed and resulted in further worsening of the clinical picture including seizures. In patient 3 VNS was implanted together with a cystoperitoneal shunt at the age of 11 years. There was some positive effect on seizure frequency but she kept having drop attacks and myoclonic seizures with VNS at highest setting. At age 16 a new device was installed which resulted in considerable improvement. 4 years later a sudden worsening of epilepsy was seen. A microfracture of the lead between pulse generator and electrodes was found and again a new device was implanted. This induced new improvement and at the age of 21 her epilepsy is well controlled with VNS and clobazam. In patient 4 VNS was implanted at the age of 7 years. 6 months post-operatively no effect (nor negative nor positive) on her epilepsy could be found.

Comments reviewer, prof Van Nieuwenhuizen:

Because of the rareness of AS no substantial trials on effects of treatment can be found. This paper also deals with only a few cases. In one patient a convincing effect of CC was demonstrated. In the other case treatment outcome in VNS plus CC or in VNS alone is less clear or even negative. Patient 3 is interesting while the effect of critical analysis of the device revealed a technical failure which could be corrected for the benefit of the patient.

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