DOUBLE CORTEX SYNDROME - ELECTROCLINICAL STUDY OF 3 CASES

We describe three female patients (aged 10, 11 and 21 years) with a Ma gnetic Resonance appearance of band heterotopia, a diffuse neuronal mi gration disorder, also known as double cortex syndrome. The clinical p icture was characterized by the association of epilepsy and mental ret ardation in all three cases, as has been previously described in patie nts with double cortex syndrome. The epileptic syndrome (Lennox-Gastau t syndrome in one case, and symptomatic partial epilepsy in the other two), the response to medical treatment, and the severity of mental re tardation were markedly different in the three patients. No clear-cut relationship was found between the clinical picture and the severity o f the neuronal migration disorder, as revealed by magnetic resonance i maging. In the three cases, EEG shares some common features: multifoca l epileptic activity with frequent bilateral diffusion, and high-ampli tude anterior fast activity, intermingled in two patients with bursts of repetitive spikes.