Surgical Treatment of Mesial Temporal Lobe Epilepsy in a Patient with Neurofibromatosis Type 1

Abstract

Neurofibromatosis type 1 (NF1) is the most common neurocutaneous disease. Main neurologic manifestations are represented by neoplasms such as optic gliomas, but epilepsy can occur by CNS lesions in less than 10%. Our patient was diagnosed, based on cafe-au-lait spots and axillary freckles. Her brain MRI did not show only multiple CNS lesions, like hamartomas, but also mesial temporal lesions. On brain-MRI the mesial temporal lesions had increased high signal intensities and suspiciously increased volume. Moreover scalp lesions could be confirmed by thin-slice, fat suppression, enhanced lesions on skin and subcutaneous areas, indicating neurofibromas. The seizure onset zone was confirmed by presurgical evaluation including electrophysiology and neuroimaging. Her seizures were refractory to multiple antiepileptic drugs, but became free after anteromesial temporal resection. We report a patient with the NF1 in mesial temporal lobe epilepsy. Epilepsy surgery was also effective in this case.