Atypical Lennox-Gastaut syndrome successfully treated with removal of a parietal dysembryoplastic tumour

Abstract

Focal brain lesions may be associated with the atypical form of Lennox-Gastaut syndrome (LGS). We describe a drug resistant LGS patient with daily seizures and a left parietal dysembryoplastic neuroepithelial tumour. Pre-surgical evaluation showed, in addition to diffuse paroxysmal EEG discharges associated with atonic and tonic axial seizures, lateralizing EEG and clinical signs pointing to left hemisphere origin of the seizures. The patient was treated with lesionectomy and after 12 months of follow-up is still seizure free. This case suggests that in patients with LGS and focal lesions the possibility of correct identification of the epileptogenic zone using anatomo-electro-clinical correlations may be the key element for 'curative' surgery.