Histopathologic findings in 37 cases of functional hemispherectomy

Hemispherectomy procedures are performed in patients for whom focal cortical resection would be predicted to produce a significant reduction in seizures. The functional hemispherectomy procedure consists of disconnecting the hemispheres while attempting, in some cases, to preserve parenchyma. This study retrospectively reviews the histopathologic findings in 37 cases of functional hemispherectomy performed between 1990 and 1998 at a major epilepsy center. Procedures were performed in 20 males and 17 females who ranged in age from 3 months to 37 years (mean age, 9.6 years). In all but two cases, more than half or all the material submitted for pathologic testing was examined histologically. Cortical dysplasias or hemimegalencephaly were identified in 14 patients. The most common patterns of dysplasia observed included architectural disorganization (n = 13), increased molecular layer neurons (n = 11), and neuronal cytomegaly (n = 11). One patient was known to have epidermal nevus syndrome. Six patients had Sturge-Weber syndrome. Remote infarct/ischemic damage was identified as the etiology of seizures in six patients; four of these patients had mild associated secondary cortical architectural abnormalities. Three patients demonstrated pathology consistent with Rasmussen's encephalitis; one additional patient had chronic encephalitis changes, not otherwise specified. In two cases, changes consistent with hippocampal sclerosis were identified; additionally, hippocampal neuronal loss and gliosis was focally identified in three patients. Most of these patients had coexistent cortical dysplasia or radiographic evidence of remote infarct. One specimen demonstrated areas of infarct following resection of an arteriovenous malformation. In two specimens, significant histopathologic findings were not identified; both of these patients had radiographic evidence of remote infarct. The spectrum of pathologic conditions that may be encountered in the setting of a functional hemispherectomy is varied and in this study most frequently included cortical dysplasia, Sturge-Weber syndrome remote infarct, and Rasmussen's encephalitis.