Background

Segmental arterial mediolysis (SAM) is a rare arterial pathology and can cause rupture or dissection of the intracranial arterial wall. The etiology is unveiled, but vasospastic stimuli such as migraine are considered as a possible cause of SAM. We present the first case of subarachnoid hemorrhage (SAH) due to SAM associated with Crohn's disease and migraine, and discuss the possible contribution of Crohn's disease to the development of SAM besides migraine.

Case description

A 33-year-old man with Crohn's disease, which had been treated with adalimumab, repeatedly underwent 3-tesla magnetic resonance (MR) imaging and angiography for severe headache due to migraine and the subsequent development of fatigue in the left arm and both legs. At 7 months after the last MR imaging studies showing no abnormalities, he had a sudden onset of severe SAH, which was caused by rupture or dissection of the terminal portion in the right internal carotid artery. As his brain-stem reflexes were absent, the patient was conservatively treated and died 6 days after the ictus. By postmortem histopathological examination, SAM was diagnosed as the cause of SAH. Vasa vasorum was also observed around the rupture point.

Conclusions

Our case suggests that: 1) the formation of vasa vasorum may be an antecedent pathology for vessel rupture of the fragile arterial wall affected by SAM, and 2) vasospastic nature of both Crohn's disease and migraine may contribute to the development of intracranial SAM.

中文翻译：

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克罗恩病相关节段性动脉中间溶解导致致命性蛛网膜下腔出血一例

背景

节段性动脉介质溶解 (SAM) 是一种罕见的动脉病变，可导致颅内动脉壁破裂或夹层。病因尚未明确，但偏头痛等血管痉挛刺激被认为是 SAM 的可能原因。我们介绍了首例由 SAM 引起的与克罗恩病和偏头痛相关的蛛网膜下腔出血 (SAH) 病例，并讨论了克罗恩病除了偏头痛之外对 SAM 发展的可能贡献。

案例描述

一名 33 岁的克罗恩病男性曾接受阿达木单抗治疗，因偏头痛导致的严重头痛以及随后出现左臂和双腿疲劳，多次接受 3 特斯拉磁共振 (MR) 成像和血管造影术. 在最后一次 MR 成像检查显示无异常后 7 个月时，他突然发作了严重的 SAH，这是由右侧颈内动脉末端部分的破裂或夹层引起的。由于脑干反射消失，患者接受保守治疗，并在发作后 6 天死亡。通过死后组织病理学检查，SAM被诊断为SAH的病因。在破裂点附近也观察到血管滋养管。

结论

我们的案例表明：1) 血管滋养管的形成可能是受 SAM 影响的脆弱动脉壁血管破裂的先行病理，2) 克罗恩病和偏头痛的血管痉挛性质可能有助于颅内 SAM 的发展。