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 adjacent 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 regarding the epileptogenicity of the lesion. is similar to non-neoplastic arachnoidal cap cells. Pathologically, meningioangiomatosis is marked by a proliferation of blood vessels collared by bland, often spindled meningothelial cells. Intervening brain parenchyma is marked by gliosis. Occasional cases of neurofibrillary tangles in intervening cortical neurons have been described [2, 3, 4]. Only a few reported cases of meningioangiomatosis have suggested the presence of coexistent focal cortical dysplasia [5, 6]. We report a case of meningioangiomatosis arising in a 23-year-old male outside the setting of Neurofibromatosis Type II. The lesion was marked by numerous neurofibrillary tangles in intervening cortical neurons and adjacent focal cortical dysplasia. The potential implications of both of these findings in meningioangiomatosis are discussed.