Sasayama Daimei, Shimojima Yasuhiro, Gono Takahisa, Kaneko Kazuma, Matsuda Masayuki, Ikeda Shu-ichi
Third Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan.
Clin Rheumatol. 2007 Oct;26(10):1761-3. doi: 10.1007/s10067-006-0502-0. Epub 2007 Jan 5.
We report a young female patient with Henoch-Schönlein purpura (HSP) nephritis complicated by reversible posterior leukoencephalopathy syndrome (RPLS). The patient suddenly showed generalized seizures and cortical blindness with severe hypertension due to renal insufficiency approximately 1 year after cessation of corticosteroid treatment for HSP nephritis. Magnetic resonance imaging (MRI) demonstrated bilateral abnormal signals mainly in the cerebellum and white matter of the occipital lobe. Clinical symptoms quickly improved in conjunction with disappearance of abnormal signals on brain MRI after starting control of hypertension and continuous hemodiafiltration with steroid pulse therapy and plasmapheresis. RPLS may be caused by vasculitis and also by hemodynamic change due to severe hypertension in HSP, particularly in patients with nephropathy. In such cases intensive treatment should be performed as soon as possible to avoid neurological sequelae.
我们报告了一名患有过敏性紫癜(HSP)肾炎并伴有可逆性后部白质脑病综合征(RPLS)的年轻女性患者。该患者在因HSP肾炎停用皮质类固醇治疗约1年后,由于肾功能不全突然出现全身性癫痫发作和皮质盲,并伴有严重高血压。磁共振成像(MRI)显示双侧异常信号主要位于小脑和枕叶白质。在开始控制高血压并采用类固醇冲击疗法和血浆置换进行持续血液透析滤过后,临床症状迅速改善,同时脑MRI上的异常信号消失。RPLS可能由血管炎引起,也可能由HSP中严重高血压导致的血流动力学变化引起,尤其是在肾病患者中。在这种情况下,应尽快进行强化治疗以避免神经后遗症。