Abstracts

Rationale: Mesial temporal lobe epilepsy with hippocampal sclerosis constitutes a distinct clinical syndrome with variable etiopathogenesis. Association with cortical dysplasia in temporal polar cortex is common, etiology and significance of this finding remains controversial. Familial occurrence of hippocampal sclerosis associated with focal cortical dysplasia of the temporal pole has not been yet described.Methods: We report on a case of two siblings (male and female) operated on for medically intractable mesiotemporal lobe epilepsy. Clinical pre-operative data and histopathological findings were compared head-to-head.Results: In both patients dual pathology in the left temporal lobe - hippocampal sclerosis associated with focal cortical dysplasia type 1b – has been histopathologically identified. When the clinical data from pre-operative investigation were compared similar results in most of them were revealed; unprovoked seizures started at the age of nine to ten with similar seizure semiology: aura consisting of unidentifiable feeling – complex partial seizure with early oral automatisms - right face clonic seizure - secondary generalization; interictal EEG showed left anterotemporal spikes and intermittent slow; ictal EEG rhythmical pattern 5-6 Hz over left fronto-temporal region; MRI revealed left hippocampal atrophy and increase in signal intensity on T2 weighted images, the increase was present also in white matter of left temporal pole with blurring of grey-white matter junction; PET showed left temporal hypometabolism and neuropsychological testing verbal memory deficit with mild frontal executive dysfunction. Both patients were left speech dominant with mild signs of bilateral speech in perception, the male was left-handed and female ambidextrous. A difference was found in medical history – both patients had febrile seizures but in different age – in five years in female and in one year of life in male. The female had history of early postnatal pneumonia; the male had history of mild perinatal distress and head trauma followed by immediate right-sided convulsions in the age of one year. The male had been on AED prophylaxis until the age of eight and then was withdrawn, in both AED was started with reoccurrence of spontaneous seizures. In spite of the medication they both had several complex partial seizures per month and several secondary generalized seizures per year. The female had surgery in the age of 22, the male in the age of 25. Following surgery they are both seizure free on follow-up shorter than one year.Conclusions: Focal cortical dysplasia may be present in familial temporal lobe epilepsy associated with hippocampal sclerosis and in such a case may play a role in etiopathogenesis of epilepsy.