Neuropathologic study of resected cerebral tissue from patients with infantile spasms

Studies to date on the neuropathologic substrates of infantile spasms have largely utilized autopsy material of children who die after a long and complicated seizure history. This makes the interpretation of primary versus secondary changes in the cerebral tissue difficult if not impossible. We have recently had the opportunity to review the neuropathologic changes in cortical tissue resected from infants and children with a history of infantile spasms. The major identifiable abnormalities were destructive lesions, sometimes classifiable as cystic-gliotic encephalomalacia, and dysplastic changes of varying degree. The cortical dysplasias had some similarity to cerebral changes described in tuberous sclerosis, including the presence of bizarre gemistocytic "balloon" cells, and secondary cytoskeletal changes within neuronal cell bodies. Such material provides an opportunity to apply immunohistochemical and molecular techniques to epileptic tissue in an attempt to understand the morphologic substrates of infantile spasms and other types of generalized epilepsy.