Shomura Keijiro, Miyashita Katsuyoshi, Kudo Seiya, Nambu Iku, Tohma Yasuo
Department of Neurosurgery, Fukui Prefectural Hospital, Fukui, Japan.
Surg Neurol Int. 2025 May 30;16:216. doi: 10.25259/SNI_207_2025. eCollection 2025.
Cerebral proliferative angiopathy (CPA) is a rare vascular disease characterized by nonfocal angiogenic activity. Numerous case reports have been published; however, despite there are a few reported cases of infratentorial CPA (or cerebellar proliferative angiopathy), no comprehensive review of this condition has been conducted.
We report two cases of infratentorial CPA, and both of them presented intracranial hemorrhage. The first case was a 48-year-old woman with an incidentally detected vascular abnormality in the cerebellum, which remained stable for 11 years before presenting with subarachnoid hemorrhage. The second case was a 5-year-old girl who presented with a sudden headache and decreased consciousness. Digital subtraction angiography was performed in both cases, but the source of hemorrhage remained unidentified, and conservative management was chosen.
A review of previously reported infratentorial CPA cases suggests that infratentorial CPA has a significantly higher risk of hemorrhage than supratentorial CPA. However, the risk of rebleeding appears to be low. Our findings highlight the need for further research to determine the optimal management strategies for this rare entity.
脑增生性血管病（CPA）是一种罕见的血管疾病，其特征为非局灶性血管生成活动。已有大量病例报告发表；然而，尽管有少数幕下CPA（或小脑增生性血管病）的报告病例，但尚未对这种情况进行全面综述。
我们报告了两例幕下CPA病例，两例均出现颅内出血。第一例是一名48岁女性，小脑偶然发现血管异常，在出现蛛网膜下腔出血前11年一直保持稳定。第二例是一名5岁女孩，出现突发头痛和意识减退。两例均进行了数字减影血管造影，但出血来源仍未明确，遂选择保守治疗。
对先前报道的幕下CPA病例的综述表明，幕下CPA的出血风险明显高于幕上CPA。然而，再出血风险似乎较低。我们的研究结果强调需要进一步研究以确定针对这种罕见疾病的最佳管理策略。
