Abstracts

Rationale: Ulegyria refers to a cerebral cortex scarring which results from a perinatal ischemic brain injury. It presents with a characteristic gyral pattern: small circumvolutions with atrophy at sulci bottom and spared apex. Ulegyria is frequently associated with epilepsy, cerebral palsy and mental disability. We aimed to analyse electro-clinical and MRI features in a subgroup of patients with pericentral ulegyria and epilepsy.Methods: We reviewed 8 patients (6 men / 2 women) with unilateral pericentral ulegyria and epilepsy from the large database of our Departments. Patients were clinically examined, underwent high resolution MRI (1.5 or 3T), EEG recordings and neuropsychological testing. Results: Mean age of patients was 38 years (27-55 years) at the reassessment time. The majority (6/8) had history of perinatal asphyxia. Seven patients had delayed motor developmental milestones and permanent hemiparesis of various severity. Mean age at seizure onset was 5.8 years (1-27 years). All patients had medically intractable epilepsy with predominantly sensory-motor seizures. However, 4/8 patients had ipsilateral hippocampal sclerosis in MRI and exhibited clinical features of temporal lobe epilepsy. Four patients had frequents episodes of either focal motor or generalised convulsive status epilepticus. All patients had lesions affecting mainly pre- and postcentral gyri; in 5/8 patients, the lesions extended to frontal and parieto-occipital areas. On MRI, gyral abnormality was associated with subcortical white matter volume loss and increased T2 and FLAIR signal. Conclusions: Patients with pericentral ulegyria have medically intractable seizures and frequent episodes of status epilepticus. Half of these patients exhibit features of temporal lobe epilepsy and harbour ipsilateral hippocampal sclerosis.