Meningioangiomatosis

Last revised by Eid Kakish on 6 Jun 2024

Meningioangiomatosis is a rare meningovascular hamartomatous plaque-like or mass-like cortical lesion extending to the overlying leptomeninges (crosses the boundary between intra- and extra-axial lesions). Given its frequent cortical location, often patients present with seizures.

Epidemiology

Meningioangiomatosis is either sporadic or associated with neurofibromatosis type 2. Presenting signs are intractable seizures (~80%) and headache. The symptoms manifest at a young age (i.e. under 20 years) 5. Seizures have a male predilection.

Radiographic features

These are slow growing solitary or multiple cortical lesions with nodular or gyriform configuration, variable calcification, and cystic degeneration. These show minimal or no contrast enhancement.

  • 90% located in the cortex, 10% located in the leptomeninges
  • 32% of lesions are frontal, 31% are temporal 5

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