Karakis Ioannis, Nuccio Audrey H, Amadio Jordan P, Fountain Arthur J
Department of Neurology, Emory University School of Medicine, Atlanta, Georgia, USA.
Department of Neurology, Emory University School of Medicine, Atlanta, Georgia, USA.
World Neurosurg. 2017 Feb;98:868.e11-868.e15. doi: 10.1016/j.wneu.2016.12.046. Epub 2016 Dec 23.
Posterior reversible leukoencephalopathy syndrome (PRES) is linked to various etiologies, including most importantly systemic hypertension. Its association with intracranial hypotension (IH), a potential sequela of various neurosurgical procedures, is underrecognized. We report a case of lumboperitoneal shunt-induced IH resulting in PRES with the goal to increase awareness and elaborate on the potential biologic mechanism, based on the Monro-Kellie hypothesis.
A 26-year-old woman with acquired immunodeficiency syndrome and epilepsy was admitted for recurrent cryptococcal meningitis and breakthrough seizures. There was radiologic evidence of ventricular enlargement, and opening pressure on serial lumbar punctures was constantly elevated. Owing to persistently elevated, symptomatic intracranial pressure and transient relief with serial lumbar punctures, a lumboperitoneal shunt was placed. The patient subsequently had a breakthrough seizure and became encephalopathic. Repeat head imaging showed reduced ventricular size, engorged venous sinuses, and tonsillar herniation in keeping with IH, coupled with extensive white matter abnormalities in bilateral parieto-occipital lobes indicative of PRES. The patient had an emergent programmable valve placed in the lumboperitoneal shunt to prevent excessive cerebrospinal fluid drainage, leading to clinical and radiologic improvement. Subsequent cerebrospinal fluid leak resulted in recurrent presentation.
IH appears to be a distinct cause of PRES not previously reported in the neurosurgical literature. It occurs in susceptible patients, on average 1-5 days after the IH trigger, and seems clinically and radiologically similar to more common hypertensive cases in terms of initial presentation and prognosis. Increased vigilance is required for prompt recognition and management.
后部可逆性白质脑病综合征（PRES）与多种病因相关，其中最重要的是系统性高血压。它与颅内低压（IH）的关联尚未得到充分认识，而颅内低压是各种神经外科手术的潜在后遗症。我们报告一例因腰大池腹腔分流术引起颅内低压导致PRES的病例，目的是提高认识，并基于Monro-Kellie假说阐述潜在的生物学机制。
一名患有获得性免疫缺陷综合征和癫痫的26岁女性因复发性新型隐球菌性脑膜炎和突破性癫痫发作入院。有影像学证据显示脑室扩大，连续腰椎穿刺的开放压力持续升高。由于颅内压持续升高且有症状，以及连续腰椎穿刺可短暂缓解症状，遂放置了腰大池腹腔分流管。患者随后发生突破性癫痫发作并出现脑病。重复头颅影像学检查显示脑室大小减小、静脉窦充血和扁桃体疝，符合颅内低压表现，同时双侧顶枕叶广泛白质异常，提示为PRES。患者在腰大池腹腔分流管中紧急放置了可调节瓣膜，以防止脑脊液过度引流，从而使临床和影像学表现得到改善。随后脑脊液漏导致病情复发。
颅内低压似乎是PRES的一个独特病因，此前神经外科文献中未见报道。它发生在易感患者中，平均在引发颅内低压后1 - 5天出现，在初始表现和预后方面，临床和影像学表现似乎与更常见的高血压病例相似。需要提高警惕以实现及时识别和处理。
