Lai Pui Man Rosalind, Ng Isaac, Gormley William B, Patel Nirav J, Frerichs Kai U, Aziz-Sultan M Ali, Du Rose
Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
Neurosurgery. 2021 Jan 13;88(2):413-419. doi: 10.1093/neuros/nyaa396.
Subarachnoid hemorrhage (SAH) from an intracranial aneurysmal rupture is the most common nontraumatic etiology for SAH, but up to 15% of patients with SAH have no identifiable source.
To assess familial predisposition to spontaneous nonaneurysmal SAH (naSAH) and to evaluate whether family history affects the severity of presentation and prognosis of this condition.
We conducted a retrospective analysis of all spontaneous SAH with negative digital subtraction angiography from 2004 to 2018. Patients were divided into 2 groups: patients with first- or second-degree relatives with intracranial aneurysms and patients with no family history. Univariate and multivariate regression analyses were used to study patient presentation, radiographic patterns of hemorrhage, and clinical outcome.
A total of 100 patients met the inclusion criteria. There were no individuals with family history of naSAH. A total of 15 patients (15%) had at least one family member with an intracranial aneurysm, of which 12 (12%) presented as SAH. Patients without family history had a higher percentage of perimesencephalic presentation, whereas those with family history had a higher percentage of nonperimesencephalic SAH presentation (47% vs 13%, odds ratio [OR] 0.17 [95% CI 0.04, 0.81]).
We found a high rate of family history of intracranial aneurysms in patients who presented with naSAH. Although there was no difference in clinical outcome in patients with and without family history, there appears to be a higher percentage of nonperimesencephalic radiographic patterns of SAH in those with family history, suggesting possible different etiologies of these hemorrhages.
颅内动脉瘤破裂导致的蛛网膜下腔出血（SAH）是SAH最常见的非创伤性病因，但高达15%的SAH患者没有可识别的病因。
评估自发性非动脉瘤性SAH（naSAH）的家族易感性，并评估家族史是否影响该疾病的表现严重程度和预后。
我们对2004年至2018年所有数字减影血管造影阴性的自发性SAH进行了回顾性分析。患者分为两组：有一级或二级亲属患有颅内动脉瘤的患者和无家族史的患者。采用单因素和多因素回归分析来研究患者的表现、出血的影像学模式和临床结局。
共有100例患者符合纳入标准。没有naSAH家族史的个体。共有15例患者（15%）至少有一名家庭成员患有颅内动脉瘤，其中12例（12%）表现为SAH。无家族史的患者中脑周围表现的比例较高，而有家族史的患者中非中脑周围SAH表现的比例较高（47%对13%，优势比[OR]0.17[95%CI 0.04，0.81]）。
我们发现naSAH患者中颅内动脉瘤家族史的发生率很高。虽然有家族史和无家族史的患者临床结局没有差异，但有家族史的患者中SAH的非中脑周围影像学模式的比例似乎更高，提示这些出血可能有不同的病因。
