Sakai N, Kawasaki Y, Imaizumi T, Kanno S, Go H, Mitomo M, Ushijima Y, Suyama K, Ito M, Hashimoto K, Hosoya M
Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima City, Fukushima, Japan.
Clin Nephrol. 2010 Jun;73(6):482-6. doi: 10.5414/cnp73482.
Reversible posterior leukoencephalopathy syndrome (RPLS) is a distinctive clinicoradiological entity observed in a variety of clinical settings. Cyclosporine (CyA)-RPLS has been reported in a few patients with focal segmental glomerulosclerosis (FSGS); however, there had been no reports on developed RPLS after the re-administration of CyA treatment. We report two patients with FSGS who developed CyA-induced RPLS and summarize the results of a literature review for similar patients. The two patients with FSGS presented here were a 4-year-old boy and a 9-year-old boy, who presented with steroid-resistant nephrotic syndrome (NS) and were treated with CyA. The first patient developed CyA-induced RPLS at the 7th day after the start of CyA treatment, and the second patient at the 16th day after the re-start of CyA treatment. The two patients complained of a visual disorder and exhibited signs of a disturbance in consciousness and hypertension. Electroencephalography (EEG) examinations revealed a generalized slow wave pattern, and magnetic resonance imaging (MRI) disclosed an area of high signal intensity in the white matter. Subsequently, CyA was discontinued and neurological symptoms improved and recrudescence of RPLS did not occur. Our findings suggest that patients with FSGS and NS who are treated with CyA should be closely monitored for the possible onset of RPLS, presenting as a disturbance in consciousness, visual disturbances and/or convulsions.
可逆性后部白质脑病综合征(RPLS)是在多种临床环境中观察到的一种独特的临床放射学实体。少数局灶节段性肾小球硬化(FSGS)患者中曾报道过环孢素(CyA)相关性RPLS;然而,此前尚无关于再次使用CyA治疗后发生RPLS的报道。我们报告了2例发生CyA诱导的RPLS的FSGS患者,并总结了对类似患者的文献综述结果。本文介绍的2例FSGS患者分别为一名4岁男孩和一名9岁男孩,他们均表现为激素抵抗型肾病综合征(NS)并接受了CyA治疗。首例患者在开始CyA治疗后第7天发生了CyA诱导的RPLS,第二例患者在重新开始CyA治疗后第16天发生。这2例患者均主诉视觉障碍,并表现出意识障碍和高血压体征。脑电图(EEG)检查显示为广泛性慢波图形,磁共振成像(MRI)显示白质有高信号强度区域。随后,停用CyA,神经症状改善,且未出现RPLS复发。我们的研究结果表明,接受CyA治疗的FSGS和NS患者应密切监测是否可能发生RPLS,其表现为意识障碍、视觉障碍和/或惊厥。