Zheng Yuan-Yuan, Weng Xiong-Peng, Fu Fang-Wang, Cao Yun-Gang, Li Yan, Zheng Guo-Qing, Chen Wei
Department of Neurology, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Wenzhou, China.
Department of Radiology, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Wenzhou, China.
Front Neurol. 2020 Jun 23;11:591. doi: 10.3389/fneur.2020.00591. eCollection 2020.
Posterior reversible encephalopathy syndrome (PRES) is a reversible neuroradiological syndrome characterized by reversible vasogenic edema. The pathophysiological mechanism is still unclear, but PRES may be triggered by various etiologies. To date, only a few PRES cases linked to cerebrospinal fluid (CSF) hypovolemia were reported. The association between PRES and CSF hypovolemia needs to be explored. We presented a case of PRES with CSF hypovolemia as a result of an inadvertent dural puncture and reviewed the literature to identify the clinical characterization and pathophysiological mechanism of PRES following CSF hypovolemia. A total of 31 cases of PRES-CSF hypovolemia was included for analysis. The median age was 33 years, with a notable female predominance (87.1%). Fifteen patients (48.4%) didn't have either a history of hypertension nor an episode of hypertension. The most common cause of CSF hypovolemia was epidural or lumbar puncture ( = 21), followed by CSF shunt ( = 6). The median interval between the procedure leading to CSF hypovolemia and PRES was 4 days. Seizure, altered mental state, and headache were the most frequent presenting symptom. The parietooccipital pattern was most frequent (71.0%). Conservative management remains the mainstay of treatment with excellent outcomes. Three patients had a second episode of PRES. CSF hypovolemia is a plausible cause of PRES via a unique pathophysiologic mechanism including arterial hyperperfusion and venous dysfunction. Patients with CSF hypovolemia is more susceptible to PRES, which is potentially life-threatening. Given that CSF hypovolemia is a common complication of anesthetic, neurological, and neurosurgical procedures, PRES should be early considered for prompt diagnosis and appropriate management.
后部可逆性脑病综合征（PRES）是一种以可逆性血管源性水肿为特征的可逆性神经放射学综合征。其病理生理机制尚不清楚，但PRES可能由多种病因引发。迄今为止，仅有少数与脑脊液（CSF）低血容量相关的PRES病例报道。PRES与CSF低血容量之间的关联有待探索。我们报告了1例因意外硬膜穿刺导致CSF低血容量的PRES病例，并回顾文献以确定CSF低血容量后PRES的临床特征和病理生理机制。共纳入31例PRES - CSF低血容量病例进行分析。中位年龄为33岁，女性占显著优势（87.1%）。15例患者（48.4%）既无高血压病史也无高血压发作史。CSF低血容量最常见的原因是硬膜外或腰椎穿刺（n = 21），其次是CSF分流（n = 6）。导致CSF低血容量的操作与PRES之间的中位间隔时间为4天。癫痫发作、精神状态改变和头痛是最常见的首发症状。顶枕部模式最为常见（71.0%）。保守治疗仍是主要的治疗方法，效果良好。3例患者出现了第二次PRES发作。CSF低血容量通过包括动脉过度灌注和静脉功能障碍在内的独特病理生理机制，是PRES的一个合理病因。CSF低血容量患者更容易发生PRES，这可能危及生命。鉴于CSF低血容量是麻醉、神经和神经外科手术的常见并发症，应尽早考虑PRES以便及时诊断和适当处理。
