Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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October 31, 2019
Meningioangiomatosis
- Background:
- Meningioangiomatosis (MA) is a rare meningovascular malformation or hamartomatous benign lesion typically affecting the leptomeninges and cerebral cortex.
- MA may occur sporadically or in association with NF2.
- Clinical Presentation:
- The sporadic type typically presents with seizures, whereas that associated with NF2 is often asymptomatic.
- The majority of those affected are children and young adults.
- Key Diagnostic Features:
- CT shows solitary or multiple cortical lesions with nodular or gyriform configuration with variable calcification.
- MRI of the brain shows corresponding susceptibility artifact on a T2 GRE sequence or SWI sequence.
- Minimal or no contrast enhancement is observed.
- Cystic degeneration and peripheral edema or gliosis on CT and MR imaging studies may be present.
- Usually localized in the temporal or frontal lobes
- Differential Diagnoses:
- Acute hemorrhage, encephalomalacia, granulomatous meningitis, cavernous malformations, and vascular abnormalities such as Sturge-Weber syndrome
- Cortical tumors such as oligodendroglioma, ganglioglioma, dysembryoplastic neuroepithelial tumor (DNET), metastasis, and meningioma should also be considered. Meningioma associated with MA is a rare condition mimicking invasive meningioma both pathologically and radiologically.
- Treatment:
- Treatment is surgical in patients presenting with seizure. The seizure-free rates after lesionectomy are variable, and most patients continue to require antiepileptic therapy.