The natural history of intracranial cavernous malformations
The natural history of intracranial cavernous malformations
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DOI：
10.3171/2011.3.focus1165
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发表时间：
2011-06-01
影响因子：
4.1
通讯作者：
Day, Arthur L.
中科院分区：
文献类型：
--
作者：
Gross, Bradley A.;Lin, Ning;Day, Arthur L.
关于脑海绵状血管瘤（CM）自然史的文献报道很多，其病变流行病学、出血率和出血危险因素存在很大差异。在这篇综述中，作者对 11 项自然历史研究进行了荟萃分析。总体男女比例为1：1，就诊时的平均年龄为30.6岁。总体而言，37% 的患者出现癫痫发作，36% 出现出血，23% 出现头痛，22% 出现局灶性神经功能缺损，10% 无症状。有些患者有不止一种症状。癫痫发作在幕上 CM 患者中最为常见，而局灶性神经功能缺损在幕下 CM 患者中常见。按部位划分，CM 位于大脑半球 (66%)、脑干 (18%)、基底节或丘脑 (8%)、小脑 (6%) 和其他部位（2.5% [幕上和幕下、胼胝体或岛叶的组合]）。总体而言，19% 的患者存在多个颅内 CM，9% 的患者具有放射学上明显的相关静脉发育异常。 3 项研究发现，每位患者每年的总体年度出血率为 2.4%（范围 1.6%-3.1%）。既往出血史和女性是出血的危险因素，而 CM 大小和多重性并不影响出血率。尽管不影响出血率本身，但深部位置是增加临床侵袭性的危险因素。 （DOI：10.3171/2011.3.FOCUS1165）
Literature reports on the natural history of cerebral cavernous malformations (CMs) are numerous, with considerable variability in lesion epidemiology, hemorrhage rates, and risk factors for hemorrhage. In this review, the authors performed a meta-analysis of 11 natural history studies. The overall male-to-female ratio was 1: 1, and the mean age at presentation was 30.6 years. Overall, 37% of patients presented with seizures, 36% with hemorrhage, 23% with headaches, 22% with focal neurological deficits, and 10% were asymptomatic. Some patients had more than one symptom. Seizure presentation was most prevalent among supratentorial CMs, while focal neurological deficits were common in patients with infratentorial CMs. By location, CMs were in the cerebral hemispheres (66%), brainstem (18%), basal ganglia or thalamus (8%), cerebellum (6%), and other (2.5% [combined supra-and infratentorial, callosal or insular]). Overall, 19% of patients harbored multiple intracranial CMs, and 9% had radiographically apparent associated developmental venous anomalies. An overall annual hemorrhage rate of 2.4% per patient-year (range 1.6%-3.1%) was identified across 3 studies. Prior hemorrhage and female sex were risk factors for bleeding, while CM size and multiplicity did not affect hemorrhage rates. Although not impacting the hemorrhage rate itself, deep location was a risk factor for increased clinical aggressiveness. (DOI: 10.3171/2011.3.FOCUS1165)