Department of Radiology, University of Minnesota, Minneapolis, MN.
Faculty of Medicine, University of Minnesota, Minneapolis, MN.
J Neuroimaging. 2018 Sep;28(5):535-541. doi: 10.1111/jon.12526. Epub 2018 May 24.
Posterior reversible encephalopathy syndrome (PRES) and acute toxic leukoencephalopathy (ATL) are both potentially reversible clinicoradiologic entities. Although their magnetic resonance imaging (MRI) findings differ, rarely both may occur simultaneously in acutely encephalopathic patients. Our aim was to determine the incidence and causes of concomitant "ATL-PRES."
Retrospective search of suspected acutely encephalopathic adults since 1998 throughout our picture archiving and communication system revealed 167 patients with PRES and 106 patients with ATL. Images of these patients were retrospectively evaluated by two neuroradiologists and a fellow to identify the cases which carry both features of PRES and ATL. Imaging findings were scored based on previously reported scoring system as mild, moderate, and severe. The clinical outcome of the patients was determined according to the modified Rankin scale.
Our search revealed a series of 6 patients (%2.2) in 273 patients who presented acutely with either encephalopathy or seizures, caused by various etiologies, including immunosuppression following transplantation (n = 2), hypertensive crisis (n = 2), chemotherapy (n = 1), and sepsis (n = 1). MRI demonstrated findings consistent with both PRES and ATL simultaneously on FLAIR and diffusion weighted imaging. Severity of imaging findings of concomitant "ATL-PRES" was concordant with each other (rho ≈ 1.0, P < .00001), and each patient eventually returned to clinical baseline. This finding, along with their similar etiologies, raises the possibility of an underlying common pathophysiologic thread, perhaps being endothelial toxicity.
Concomitant "ATL-PRES" was found in 2.2% of the patients in a large cohort of ATL and PRES. Etiologies varied. Clinical symptoms and MRI findings were potentially reversible.
后部可逆性脑病综合征(PRES)和急性中毒性脑白质病(ATL)都是潜在可逆转的临床放射学实体。尽管它们的磁共振成像(MRI)表现不同,但在急性脑病患者中很少同时出现两种情况。我们的目的是确定同时发生的“ATL-PRES”的发生率和原因。
通过我们的图像存档和通信系统,对 1998 年以来疑似急性脑病的成年人进行回顾性搜索,共发现 167 例 PRES 患者和 106 例 ATL 患者。由两位神经放射科医生和一位研究员对这些患者的图像进行回顾性评估,以确定同时具有 PRES 和 ATL 特征的病例。根据先前报道的评分系统对成像结果进行评分,分为轻度、中度和重度。根据改良 Rankin 量表确定患者的临床结局。
我们的搜索结果显示,在 273 例因各种病因(包括移植后免疫抑制 2 例、高血压危象 2 例、化疗 1 例和败血症 1 例)而出现急性脑病或癫痫发作的患者中,有 6 例(2.2%)存在同时符合 PRES 和 ATL 的影像学表现。FLAIR 和弥散加权成像显示同时存在 PRES 和 ATL 的影像学表现。同时发生的“ATL-PRES”的影像学表现严重程度彼此一致(rho ≈ 1.0,P<.00001),每个患者最终都恢复到临床基线。这一发现以及它们相似的病因提示可能存在潜在的共同病理生理线索,也许是内皮毒性。
在大型 ATL 和 PRES 队列中,2.2%的患者同时存在“ATL-PRES”。病因各异。临床症状和 MRI 表现可能是可逆的。