Kono Yu, Nakamori Tomoki, Imafuku Ichiro, Kunimoto Masanari, Takei Miki, Kusaba Tokuo
Department of Neurology, Yokohama Rosai Hospital.
Rinsho Shinkeigaku. 2005 Apr;45(4):317-20.
We report on a 31-year-old gravid woman with reversible posterior leukoencephalopathy syndrome (RPLS) associated with HELLP syndrome. The patient was hospitalized and underwent an emergency cesarean section at the 34th week of pregnancy due to severe toxemia. After labor her blood pressure acutely increased to 180/100 mmHg and a generalized convulsion occurred. Laboratory data revealed hemolysis, elevated liver enzymes, and low platelets, consistent with HELLP syndrome. FLAIR and Apparent Diffusion Coefficient images on MRI showed hyperintense lesions bilaterally in the posterior white matter, however Diffusion-Weighted images revealed slightly hyperintense changes in the same locations. With the use of diltiazem for blood pressure control, and anticonvulsants, convulsions disappeared and consciousness level improved. Moreover the MRI abnormalities also improved after therapy. This case was diagnosed as RPLS associated with HELLP syndrome and is the first case of an investigation utilizing Diffusion-Weighted and Apparent Diffusion Coefficient images to establish this diagnosis. Based on these results, possible mechanisms of RPLS may be vasogenic edema mediated by a cerebrovascular endothelial disturbance of cerebral vessels and a rapid blood-pressure increase due to HELLP syndrome.
我们报告了一名31岁的妊娠女性,患有与HELLP综合征相关的可逆性后部白质脑病综合征(RPLS)。该患者因重度子痫前期于妊娠34周住院并接受了急诊剖宫产。分娩后她的血压急剧升至180/100 mmHg,并发生了全身性惊厥。实验室检查结果显示溶血、肝酶升高和血小板减少,符合HELLP综合征。MRI的FLAIR和表观扩散系数图像显示双侧后部白质高信号病变,然而弥散加权图像显示相同部位有轻度高信号改变。使用地尔硫卓控制血压并给予抗惊厥药物后,惊厥消失,意识水平改善。此外,治疗后MRI异常也有所改善。该病例被诊断为与HELLP综合征相关的RPLS,是首例利用弥散加权和表观扩散系数图像进行诊断的研究病例。基于这些结果,RPLS的可能机制可能是脑血管内皮功能紊乱介导的血管源性水肿以及HELLP综合征导致的血压快速升高。