Agenesis of the Corpus Callosum and Aicardi Syndrome: a neuroimaging and clinical comparison

Publication date: Available online 4 January 2017
Source:Pediatric Neurology
Author(s): T. Govil-Dalela, A. Kumar, R. Agarwal, H.T. Chugani
ObjectiveAgenesis of the corpus callosum (ACC) can be seen in patients with epilepsy, either in isolation or as part of various neurologic conditions, such as Aicardi syndrome. In this study, we evaluated the clinical and neuroradiological differences between children with non-syndromic ACC and those with Aicardi syndrome.MethodsWe evaluated 31 children with epilepsy and ACC (11 males: 20 females), 14 of whom had Aicardi syndrome (all females). We compared their clinical histories, radiologic and electrophysiologic findings, treatments, and their outcome.ResultsMedian age at seizure onset was lower in the Aicardi syndrome group compared to non-syndromic ACC (2 vs 5 months, p=0.006). The developmental impairment in terms of verbalization and ambulation was significantly worse in patients with Aicardi syndrome. The extent of magnetic resonance imaging (MRI) as well as glucose metabolism positron emission tomography (PET) involvement was more extensive in children with Aicardi syndrome than in non-syndromic ACC. In both groups, the PET scan showed a much more extensive area of involvement than suggested by the MRI scan. Four children underwent epilepsy surgery with significant improvement, but were not seizure free. Outcome was worse in those with PET showing abnormalities in the non-surgical hemisphere despite normal appearance on MRI. All children who did not undergo surgery also continued to have seizures at last follow-up.ConclusionsChildren with Aicardi syndrome have earlier seizure onset, worse developmental outcome and larger areas of brain abnormalities on neuroimaging as compared to non-syndromic ACC patients. PET reveals larger area of abnormalities, compared to MRI. Although epilepsy surgery in ACC may offer some palliative benefit in seizure frequency, none of our patients became seizure free.



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