A Case of Refractory Epilepsy; Neuronal Migration Disorders - Abstract
Refractory epilepsy in childhood is a common presentation of cortical heterotopia, a form of neuronal migration disorders. Subcortical band heterotopia or double cortex syndrome, subependymal heterotopia and subcortical heterotopia are three common types of cortical heterotopia. Here we present a case of 10 year old non dysmorphic boy with static encephalopathy who was evaluated for refractory epilepsy.He had significant cognitive impairment, developmental delay, aggressive behavioral outbursts and self mutilating behavior. He started to have seizures approximately at age six month and over the years seizures evolved to different patterns representing Lennox Gastaut syndrome. His EEG reflected a slow and disorganized pattern with bitemporal slow spikes. His brain MRI revealed band cortical heterotopia without hippocampal pathology or mesial temporal lobe sclerosis. He did not have any genetic abnormality on karyotyping, chromosome micro-array or whole exome sequencing. He was dignosed with double cortex syndrome based on clinioradiological findings without any identifiable genetic abnormality. This case reportalong with review of literature reinforce the different spectrum of clinical manifestations of different types of cortical heterotopia.