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过敏性紫癜和溶血尿毒综合征中的后部可逆性脑病综合征

Posterior Reversible Encephalopathy Syndrome in Henoch-Schonlein Purpura and Hemolytic Uremic Syndrome.

作者信息

Fidan Kibriya, Kandur Yasar, Ucar Murat, Gucuyener Kivilcim, Soylemezoglu Oguz

机构信息

Department of Pediatric Nephrology, Faculty of Medicine, Gazi University, Ankara, Turkey.

Department of Radiology, Faculty of Medicine, Gazi University, Ankara, Turkey.

出版信息

J Clin Med Res. 2016 Jul;8(7):544-7. doi: 10.14740/jocmr2157w. Epub 2016 May 29.

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological syndrome, composed of symptoms such as headache, seizures, visual disturbances, lethargy, confusion, stupor, focal neurologic findings and radiological findings of bilateral gray and white matter abnormalities suggestive of edema in the posterior regions of the cerebral hemispheres. PRES is associated with significant morbidity and mortality if it is not expeditiously recognized. Magnetic resonance image (MRI) represents the most sensitive imaging technique for recognizing PRES. PRES has been seen in various clinical settings including renal disorders such as acute glomerulonephritis, lupus nephritis, nephrotic syndrome, and drug usage such as calcineurin inhibitors. We aimed to present two study cases for such clinical setting. In this report, we present two patients with PRES in whom the primary diagnosis was hemolytic uremic syndrome (HUS) and Henoch-Schonlein purpura (HSP). Both of them were treated with anticonvulsant and proper antihypertensive drugs. A repeated MRI scan of the head, an ophthalmologic assessment, and a follow-up electroencephalogram produced normal results with no sequelae. Early recognition of PRES as a complication during different diseases and therapies in childhood may facilitate the appropriate treatment, so that intensive treatment should be performed as soon as possible to avoid neurological sequelae.

摘要

后部可逆性脑病综合征(PRES)是一种临床放射学综合征,由头痛、癫痫发作、视觉障碍、嗜睡、意识模糊、昏迷、局灶性神经学表现以及双侧灰白质异常的放射学表现(提示大脑半球后部水肿)等症状组成。如果不能迅速识别,PRES会导致显著的发病率和死亡率。磁共振成像(MRI)是识别PRES最敏感的成像技术。PRES可见于各种临床情况,包括肾脏疾病,如急性肾小球肾炎、狼疮性肾炎、肾病综合征,以及药物使用情况,如钙调神经磷酸酶抑制剂。我们旨在介绍针对这种临床情况的两个研究病例。在本报告中,我们介绍了两名PRES患者,其初步诊断分别为溶血尿毒综合征(HUS)和过敏性紫癜(HSP)。他们均接受了抗惊厥药物和适当的降压药物治疗。头部的重复MRI扫描、眼科评估以及随访脑电图均产生了正常结果,且无后遗症。在儿童期不同疾病和治疗过程中早期识别PRES作为一种并发症,可能有助于进行适当的治疗,因此应尽快进行强化治疗以避免神经后遗症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2ba/4894025/c6244f270d02/jocmr-08-544-g001.jpg

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