Purpose: 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 analysed electro-clinical and MRI features in patients with ulegyria and epilepsy.
Patients and Methods: We reviewed 25 patients (14m/11w) with ulegyria and epilepsy from the database (about 5000 patients with epilepsy) of our unit. Patients were clinically examined, underwent high resolution MRI, EEG recordings, PET, SPECT and neuropsychological testing. Two patients with refractory seizures underwent epilepsy surgery.
Results: Mean age of patients was 34 years (5-66 years) at the reassessment time. The majority (16/25, 64%) had a history of perinatal asphyxia. Fifteen patients had delayed developmental milestones; twenty had learning disability, five patients were severely disabled. Mean age at seizure onset was 4.2 years (1-18 years). Seventeen patients (68%) had medically intractable epilepsy. Eleven patients (44%) had occipital lobe seizures. The majority (n=24, 96%) had parieto-occipital lesions on MRI. In thirteen patients (52%) ulegyria was bilateral. Twelve patients (48%) had hippocampal sclerosis. Two patients underwent epilepsy surgery with an excellent postoperative outcome (Engel, class I A and C).
Conclusion: Patients with ulegyria often have a history of perinatal asphyxia and present with pharmacoresistant seizures. Their presurgical assessment is complicated due to frequent dual pathology (hippocampal sclerosis) and bilateral lesions.
- epilepsy surgery
- mental retardation
- perinatal asphyxia