Department of Neurology, The University of Texas at Austin Dell Medical School, Austin, Texas.
Division of Neurology, Department of Pediatrics, University of California San Francisco, San Francisco, California.
Semin Neurol. 2020 Jun;40(3):294-302. doi: 10.1055/s-0040-1702942. Epub 2020 Feb 20.
Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical-radiologic diagnosis that affects children and adolescents, but it is much more frequently reported in adults. Clinically, patients present with severe and commonly recurrent thunderclap headaches. Typical precipitating triggers include vasoactive substances, serotonergic agents, and the postpartum period. There may be associated neurologic complications at presentation or in the weeks following, such as convexity subarachnoid hemorrhage, stroke, cerebral edema, cervical artery dissection (CeAD), and seizures. Angiographically, the cerebral arteries demonstrate segmental vasoconstriction and dilation, although imaging early in the clinical course may be normal. Work-up is performed to exclude intracranial disorders such as vasculitis, subarachnoid hemorrhage due to ruptured aneurysm, meningitis, and intracranial venous sinus thrombosis. Within 1 month of initial symptom onset, clinical symptoms such as severe headache have ceased, and within 3 months, the cerebral vasoconstriction is much improved or resolved. Management involves avoidance of precipitating triggers and potentially short-term pharmacotherapy with calcium channel blockers for patients with associated neurologic complications. Steroids are not recommended and may worsen the clinical outcome. Prognosis is excellent in the large majority of patients, and only 5% of patients experience a recurrence of RCVS.
可逆性脑血管收缩综合征（RCVS）是一种临床-放射学诊断，可影响儿童和青少年，但在成年人中更为常见。临床上，患者表现为严重且常反复发作的霹雳头痛。典型的诱发因素包括血管活性物质、5-羟色胺能药物和产后。在出现时或在随后的几周内可能会出现相关的神经系统并发症，如大脑凸面蛛网膜下腔出血、中风、脑水肿、颈内动脉夹层（CeAD）和癫痫。血管造影显示脑动脉节段性收缩和扩张，尽管在临床病程早期影像学可能正常。进行检查以排除颅内疾病，如血管炎、破裂动脉瘤引起的蛛网膜下腔出血、脑膜炎和颅内静脉窦血栓形成。在初始症状出现后 1 个月内，严重头痛等临床症状已经停止，在 3 个月内，脑血管收缩明显改善或消退。治疗包括避免诱发因素和潜在的短期钙通道阻滞剂治疗伴有神经系统并发症的患者。不建议使用类固醇，因为它可能会使临床预后恶化。在绝大多数患者中预后良好，只有 5%的患者会出现 RCVS 复发。
