Seizure freedom following 49 years of refractory epilepsy due to focal cortical dysplasia

Mr Paul Harder, a 66 year-old right-handed male architect was born after an uneventful pregnancy and suffered no childhood diseases before developing epilepsy at age 7 years. Initially, seizures consisted of episodes of the right hand and arm shaking. He was treated with numerous antiepileptic drugs throughout childhood and adolescence, with a maximum interval between seizures of 2 years, aged 15 to 17, with no concurrent change in his antiepileptic drugs (AEDs). Following this, however, his epilepsy remained refractory to medical treatment. Habitual seizures occurred during wake and sleep, and had always been stereotyped. Seizures consisted of an initial indescribable feeling in his head and/or moving up the right arm followed by hypermotor movements of the right arm, sometimes spreading to the right leg and the whole of the right side. Seizure duration was always 10–30 s, and movements were large amplitude, ‘jerky’ and non-purposeful with no posturing. Nocturnal seizures sometimes featured alteration in awareness, but there was always rapid recovery with no postictal confusion. Throughout most of his adult life, he received high doses of phenytoin (PHT 600 mg/day) and carbamazepine (CBZ 1400–1600 mg/day) without seizure control from the sequential addition of valproate, primidone, clonazepam, …