Meningioangiomatosis: A review of the variable manifestations and complex pathophysiology

Abstract

Meningioangiomatosis (MA) is a rare, complex and heterogeneous disease of meningovascular proliferation that is found primarily in the leptomeninges and cerebral cortex but can involve subcortical white matter and other brain regions such as the cerebellum and deep gray matter. MA may be found in pediatric or adult populations and may be sporadic or neurofibromatosis-associated. The presentation of MA is highly variable: it may be associated with other neurological diseases; clinically presents on a spectrum from asymptomatic to seizures or focal deficits; radiologically presents with multifocal, tumor-like, or cystic lesions, or may appear normal; and pathologically may have cellular or vascular predominance. In this article, we review the various manifestations of MA including neurofibromatosis-associated MA, multifocal MA, cystic MA, and MA associated with meningioma, other brain tumors, focal cortical dysplasia, neurodegenerative changes, and post-radiation changes. The treatment of MA is also reviewed. While the pathogenesis of MA remains elusive, we discuss the proposed theories such as developmental, dysplastic, hamartomatous or reactive ethology in given variants. It is important for physicians to be aware of MA as more research on this complex entity is needed and timely diagnosis may benefit outcomes in patients with MA.

Keywords: Focal cortical dysplasia; Meningioangiomatosis; Meningioma; Neurofibromatosis; Refractory epilepsy.