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Multiple extra-axial calcified lesions and tortuous vessels in a patient with meningioangiomatosis and executive
Mayuko Miyata1, Hiroki Sato1, Takuma Maeda1
1Department of Cerebrovascular Surgery, Saitama Medical University International Medical Center, Hidaka-shi, Saitama, Japan.
Abstract:
Meningioangiomatosis (MA) is a rare benign hamartomatous lesion characterized by leptomeningeal and cortical vascular proliferation, and the predominant imaging feature is cerebral calcification. Although the radiological findings of this entity are well known, angiographic characteristics are incompletely understood. This report describes a case of a 54-year-old woman with sporadic MA and lifelong executive dysfunction with novel phase-specific angiographic features. Computed tomography revealed diffuse calcified lesions along the right cerebral convexity. Cerebral angiography showed previously undescribed tortuous vessels in the venous phase without arteriovenous shunting. Despite extensive radiological changes, the patient's neurological status remained stable over a 4-year follow-up period without therapeutic interventions. The identification of frizzy, tortuous cortical veins in the venous phase-previously unreported in MA-serves as a practical imaging sign that can alert clinicians to this rare entity, aid in non‑invasive differentiation from other calcified cortical lesions, and potentially, avoid the need for diagnostic brain biopsy. However, long‑term clinical and imaging follow‑up is mandatory.
Insights
Meningioangiomatosis (MA) is a rare brain lesion. New angiographic findings reveal unique tortuous veins, aiding diagnosis and potentially avoiding biopsies.
Area of Science:
- Neurology
- Neuroradiology
- Vascular Malformations
Background:
- Meningioangiomatosis (MA) is a rare, benign hamartomatous lesion involving leptomeningeal and cortical vascular proliferation.
- Cerebral calcification is the predominant imaging feature, but angiographic characteristics remain incompletely understood.
Observation:
- A case of sporadic MA in a 54-year-old woman with lifelong executive dysfunction is presented.
- Computed tomography showed diffuse calcified lesions along the right cerebral convexity.
- Cerebral angiography revealed previously undescribed tortuous vessels in the venous phase without arteriovenous shunting.
Findings:
- The key finding was the identification of "frizzy," tortuous cortical veins in the venous phase, a novel angiographic sign in MA.
- These findings were observed in a patient with stable neurological status over a 4-year follow-up period without intervention.
Implications:
- The newly described "frizzy" cortical veins in the venous phase can serve as a practical imaging sign for MA.
- This sign may aid in non-invasive differentiation from other calcified cortical lesions, potentially avoiding the need for brain biopsy.
- Long-term clinical and imaging follow-up remains mandatory for patients with MA.
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