Tuberous sclerosis complex and sphenoid meningioma.

Unidad Académica de Medicina de la Universidad Autónoma de Nayarit, México; Departamento de Neuropatología. Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, México; Servicio de Neurocirugía, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, México; Servicio de Neuropsiquiatría, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, México. Tuberous sclerosis complex (TSC) is an autosomal dominant multisystem neurocutaneous syndrome, genetic disease characterized by the presence of hamartomatous lesions in multiple organs (skin, brain, heart, kidneys, liver, and lungs). Cortical tubers, subependymal nodules and subependymal giant cell astrocytoma are the characteristic intracranial lesions of TSC. However, the full triad occurs in only 29% of patients. The majority of patients with TSC have epilepsy, although the mechanisms underlying epileptogenesis remain unknown. Tubers are traditionally thought to be stable lesions that result from abnormal corticogenesis in early fetal development. Herein we report a 52-year-old woman without family history of neurofibromatosis type 2, with subependymal nodules, giant cell astrocytoma and infiltrating transitional meningioma.