Long-term outcome after epilepsy surgery for focal cortical dysplasia

Object. Reports of outcomes for surgical treatment of cortical dysplasia associated with epilepsy are conflicting due to the inclusion of patients with a wide range of malformations of cortical development. The authors report their experience and the long-term outcome for a subgroup of patients with the histopathological diagnosis of focal cortical dysplasia of Taylor. Methods. The records of 22 patients with focal cortical dysplasia of Taylor (15 with the balloon-cell type and seven with the nonballoon-cell type) were reviewed. There were 11 female and 11 male patients whose mean age was 26 +/- 17.6 years (mean +/- standard deviation [SD]) at surgery. The details of their epilepsy evaluation and resection were analyzed. Extent of resection was preoperatively planned using information obtained from long-term intracranial monitoring (15 patients) and/or more definitively determined by histopathologically proven clear margins during resection when feasible ( 12 patients). The mean duration of follow up was 6.3 +/- 5.1 years (mean +/- SD, range 0.5-15.6 years). Risk factors for epilepsy were trauma (seven patients) or meningoencephalitis (one patient) 14 patients (64%) had no obvious risk factors. The mean age at seizure onset was 9.2 years and the mean duration of their epilepsy was 16.1 +/- 9 years. In two patients there were no adverse findings on magnetic resonance (MR) imaging. In 15 patients (68%), the epileptogenic zone identified on long-term intracranial monitoring extended beyond the abnormality observed on MR images. Focal resection (lesion plus margins) was performed in 14 patients (64%), whereas eight (36%) underwent partial/tailored lobectomy. Two patients underwent multiple subpial transections in addition to partial lesionectomy because their lesions involved the sensorimotor cortex. In these two, functional MR imaging confirmed a normal functional anatomy despite the presence of the cortical dysplasia. Eleven (92%) of 12 patients who underwent resection guided by histopathologically proven clear margins and three (43%) of seven patients who underwent histopathologically proven subtotal resection have remained seizure free. Evidence of clear margins was significantly associated with an improved seizure outcome (p = 0.003). Postoperatively, expected deficits included nondisabling visual field defects, which occurred in three patients (14%), and transient sensorimotor deficits, which appeared in five (23%). Two patients had meningitis, which was successfully treated with antibiotics. Overall, 16 patients (73%) are either seizure free (13 patients), have rare nondisabling partial seizures (one patient), or had one seizure after their medication was changed (two patients). Thirteen patients (59%) have discontinued anticonvulsant medications or are being maintained on monotherapy. Of five patients (23%), two have had rare disabling seizures or significant reduction in their seizure frequency (three patients). One patient's seizures have remained the same. Conclusions. Focal cortical dysplasias are a distinct subgroup of malformations of cortical development and have a favorable outcome after resection. The epileptogenic zone often extends beyond the abnormality found on neuroimaging. Resection of the epileptogenic zone guided by histopathologically proven clear margins is associated with an improved seizure outcome.