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Meningioangiomatosis associated with focal cortical dysplasia and neurofibrillary tangles.

Meningioangiomatosis is a rare developmental lesion of probable hamartomatous origin. It is marked by a proliferation of blood vessels and meningothelial cells and is associated with chronic epilepsy. We report a case of a 23-year-old male with no evidence of Neurofibromatosis Type II who presented with meningioangiomatosis. Intracytoplasmic neurofibrillary tangles within dysplastic neurons were present throughout the lesion. Cortical tissue adjacen to the lesion demonstrated cytoarchitectural disorganization with an absence of cortical Layer II, malpositioning of cortical neurons and dysmorphic neurons consistent with a Palmini et al. focal cortical dysplasia type IIA, ILAE focal cortical dysplasia Type IIIc. The presence of coexistent focal cortical dysplasia supports a developmental nature of the meningioangiomatosis and has potential implications regardg the epileptogenicity of the lesion.