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Removal of malformation in cerebral proliferative angiopathy: illustrative case

Gwang Yoon Choi, Hyuk Jai Choi, Jin Pyeong Jeon, Jin Seo Yang, Suk-Hyung Kang, and Yong-Jun Cho

BACKGROUND

Cerebral proliferative angiopathy (CPA) is a rare vascular disorder distinct from arteriovenous malformation. Because of the disorder’s rarity, there is still a controversy on the most promising treatment method for CPA. However, several meta-analysis articles suggest indirect vascularization such as encephalo-duro-arterio-synangiosis as an effective way of treating symptoms that are medically uncontrolled.

OBSERVATIONS

The authors describe a case of an 11-year-old boy with this disease, who had epilepsy that was intractable despite conservative management. The patient recovered from his symptoms after the vascular malformation was surgically removed. This is the first reported case of surgical removal in CPA.

LESSONS

Although further investigation on the best treatment for CPA is needed, the authors believe surgical intervention may also be an effective treatment modality when a patient presents with persisting symptoms.