School of Medicine, Universidad de los Andes, Bogotá, Colombia.
Department of Neurology, Hospital Universitario Fundación Santa Fe, Bogotá, Colombia; School of Medicine, Universidad de los Andes, Bogotá, Colombia; School of Medicine, Universidad El Bosque, Bogotá, Colombia.
J Clin Neurosci. 2019 Dec;70:183-188. doi: 10.1016/j.jocn.2019.08.014. Epub 2019 Aug 12.
To describe patient characteristics, radiological findings and the clinical course of adults with fatal reversible cerebral vasoconstriction syndrome (RCVS).
A systematic literature search from January 1, 2000, until December 31, 2018, was performed using PubMed, EMBASE, Scopus, Cochrane reviews, LILACS and Scielo. Studies reporting RCVS in adult patients with fatal outcomes were included.
430 studies were initially identified, 179 full-text articles were reviewed, and 9 publications describing 12 subjects were included. The vast majority of the reports were from the U.S. Most of the female cases occurred during postpartum. All patients had a headache on initial presentation, although only 42% had thunderclap headache. A CT scan was performed on 67% of the patients. Imaging results were diverse, with a tendency toward cerebral hemorrhage followed by mixed cases. The main course of treatment included steroids (58% of the patients), with only 42% receiving nimodipine. The time to death ranged from 4 to 14 days, with a median of 9.2 days (SD ± 3.2).
We found that the majority of fatal cases reported in the literature are most likely related to postpartum angiopathy. We established a tendency in the onset of brain hemorrhage and the combination of infarction and brain hemorrhage. We described various markers for poor prognosis, including focal signs, the presence of hemorrhage and infarct in the first diagnostic image obtained and the need for invasive interventions. The majority of fatal cases in our report occurred in women, with over half of those cases during the puerperium period.
描述成人致死性可逆性脑血管收缩综合征（RCVS）患者的临床特征、影像学表现及临床经过。
通过 PubMed、EMBASE、Scopus、 Cochrane 图书馆、LILACS 和 Scielo 对 2000 年 1 月 1 日至 2018 年 12 月 31 日的文献进行系统性检索，纳入报道成人致命性 RCVS 患者的研究。
最初确定了 430 项研究，对 179 篇全文文章进行了回顾，纳入了 9 项描述 12 例患者的研究。大多数报告来自美国，大多数女性病例发生在产后。所有患者初始表现均有头痛，尽管仅有 42%的患者为霹雳样头痛。67%的患者行 CT 扫描。影像学结果多种多样，以脑出血为主，其次为混合性病例。主要治疗方法包括激素（58%的患者），仅有 42%的患者使用尼莫地平。死亡时间范围为 4-14 天，中位数为 9.2 天（SD ± 3.2）。
我们发现，文献中报道的大多数致死性病例很可能与产后血管病变有关。我们确定了脑出血及梗死和脑出血混合的发病倾向。我们描述了各种不良预后的标志物，包括局灶性体征、首次获得的诊断图像中存在出血和梗死，以及需要进行有创干预。我们报告的大多数致命病例发生在女性，其中一半以上发生在产后。
