Department of Radiology, Xiangya Hospital, Central South University, Changsha 410008, China.
Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2022 Dec 28;47(12):1673-1682. doi: 10.11817/j.issn.1672-7347.2022.220099.
Reversible posterior leukoencephalopathy syndrome (RPLS) is a rare clinical imaging syndrome. The causes of RPLS are complex and diverse, the pathogenesis is not yet clear. The onset is urgent and the onset age span is large, ranging from children to the elderly. The clinical symptoms of RPLS have no significant specificity, which can be manifested as headache, blurred vision, disturbance of consciousness or seizures. Clinicians have little knowledge on the disease, which may lead to misdiagnosis or missed diagnosis. This study aims to analyze and summarize the MRI changes and clinical characteristics regarding RPLS patients, so as to provide basis for rapid diagnosis and timely intervention for this disease.
The clinical data and complete imaging data of 77 patients with RPLS diagnosed in Xiangya Hospital of Central South University from January 2012 to March 2021 were retrospectively collected. The main image data include T1 weighted imaging (T1WI), T2 weighted imaging (T2WI), T2 liquid attenuation inversion recovery (T2-FLAIR), diffusion weighted imaging (DWI) (b value=1 000×10-6 mm2/s), and apparent diffusion coefficient (ADC). The case group included 63 patients who underwent DWI examination, and 71 normal controls matched in age and sex. The characteristics of patients' magnetic resonance signals and the ADC value of 19 regions of interest (ROI) were analyzed. The differences in bilateral ADC value in the case group, the difference of ADC value between the case group and the normal control group, and the difference of ADC value in the case group before and after treatment were compared.
Compared with the normal control group, the ADC value of the right frontal lobe, bilateral parietal lobe, bilateral thalamus, bilateral head of caudate nucleus, left lenticular nucleus, right internal capsule, bilateral temporal lobe and pons in the case group were significantly higher (all P<0.01). There was no significant difference in ADC value of bilateral sides of the case group and before and after treatment in the case group (all P>0.01). The lesions of RPLS were widely distributed and multiple, usually high signal in the posterior parieto temporo occipital lobe or pons of the brain, and involved the cortex and subcortical white matter. Most of them were bilateral, but not completely symmetrical.
The imaging manifestations of RPLS and the occurrence and development of clinical symptoms are basically synchronous. The imaging manifestations are specific. Magnetic resonance imaging can show the range of involvement of RPLS. ADC value can provide information on the severity of the disease and predict the prognosis. There are few reversible diseases. It is very important to fully understand and timely diagnose the disease.
可逆性后部脑白质病变综合征（RPLS）是一种罕见的临床影像学综合征。其病因复杂多样，发病机制尚不清楚。发病急，发病年龄跨度大，从儿童到老年人。RPLS 的临床症状无明显特异性，可表现为头痛、视力模糊、意识障碍或癫痫发作。临床医生对此病了解甚少，可能导致误诊或漏诊。本研究旨在分析总结 RPLS 患者的 MRI 改变和临床特征，为该病的快速诊断和及时干预提供依据。
回顾性收集 2012 年 1 月至 2021 年 3 月中南大学湘雅医院诊断为 RPLS 的 77 例患者的临床资料及完整影像学资料。主要影像资料包括 T1 加权成像（T1WI）、T2 加权成像（T2WI）、T2 液体衰减反转恢复（T2-FLAIR）、弥散加权成像（DWI）（b 值=1 000×10-6mm2/s）及表观弥散系数（ADC）。病例组 63 例行 DWI 检查，71 例年龄、性别相匹配的正常对照。分析患者磁共振信号特征及 19 个感兴趣区（ROI）的 ADC 值。比较病例组双侧 ADC 值的差异、病例组与正常对照组 ADC 值的差异、病例组治疗前后 ADC 值的差异。
与正常对照组比较，病例组右侧额叶、双侧顶叶、双侧丘脑、双侧尾状核头部、左侧豆状核、右侧内囊、双侧颞叶及脑桥 ADC 值明显升高（均 P<0.01）。病例组双侧 ADC 值及治疗前后比较差异均无统计学意义（均 P>0.01）。RPLS 的病变广泛分布，多发，常为大脑后顶颞枕叶或脑桥的高信号，累及皮质及皮质下白质。多为双侧，但不完全对称。
RPLS 的影像学表现与临床症状的发生和发展基本同步，影像学表现具有特异性。磁共振成像可以显示 RPLS 的受累范围。ADC 值能提供疾病严重程度的信息，预测预后。可逆性疾病较少，充分了解并及时诊断该病非常重要。
