Onder Ali Mirza, Lopez Robert, Teomete Uygar, Francoeur Denise, Bhatia Rita, Knowbi Obioma, Hizaji Rana, Chandar Jayanthi, Abitbol Carolyn, Zilleruelo Gaston
Department of Pediatrics, Pediatric Nephrology, West Virginia University, Morgantown, WV 26506-9214, USA.
Pediatr Nephrol. 2007 Nov;22(11):1921-9. doi: 10.1007/s00467-007-0578-z. Epub 2007 Aug 11.
Posterior reversible leukoencephalopathy syndrome (PRES) clinically presents with seizures, severe headaches, and mental and visual changes. Our goal was to describe the clinical features, triggering factors, neuro-imaging findings, and electroencephalogram (EEG) findings in a pediatric cohort with renal disease. We retrospectively analyzed the records of 18 children with the diagnosis of PRES between January 2001 and June 2006 at the University of Miami/Holtz Children's Hospital, USA. There were 22 PRES episodes. The most common clinical presentation was generalized tonic-clonic seizures in 59% (13/22). The most common identified trigger of PRES was hypertensive crisis in 59% (13/22). Almost half of the children had no evidence of on-going uncontrolled hypertension; 44% (8/18) had normal funduscopic examination findings, and 50% (9/18) had no or mild left ventricular hypertrophy. Two of the 18 patients had recurrent PRES episodes, three episodes each. Diffuse slowing was the most common finding on the EEGs. Atypical magnetic resonance imaging (MRI) findings were more prevalent in the imaged cases (62% vs 25%, P < 0.05). All the computerized tomography (CT) scans were normal, despite the positive MRI findings in four cases when both types of imaging was used. All the episodes had total clinical resolution. In conclusion, despite the diverse initial trigger, acute hypertension seems to be the common pathogenic pathway for pediatric PRES. MRI seems superior to CT, with better sensitivity due to its high resolution and diffusion-weighted imaging. The lesions do not necessarily have to be in the posterior white matter and may not be totally reversible.
后部可逆性白质脑病综合征（PRES）临床上表现为癫痫发作、严重头痛以及精神和视觉改变。我们的目标是描述一组患有肾脏疾病的儿科患者的临床特征、诱发因素、神经影像学表现和脑电图（EEG）表现。我们回顾性分析了2001年1月至2006年6月期间在美国迈阿密大学/霍尔兹儿童医院诊断为PRES的18名儿童的记录。共有22次PRES发作。最常见的临床表现是59%（13/22）的患者出现全身强直阵挛性发作。最常见的PRES诱发因素是59%（13/22）的患者出现高血压危象。几乎一半的儿童没有持续未控制高血压的证据；44%（8/18）的儿童眼底检查结果正常，50%（9/18）的儿童没有或仅有轻度左心室肥厚。18名患者中有2名出现复发性PRES发作，每人发作3次。脑电图最常见的表现是弥漫性减慢。在成像病例中，非典型磁共振成像（MRI）表现更为普遍（62%对25%，P<0.05）。尽管在4例同时使用两种成像方式时MRI呈阳性，但所有计算机断层扫描（CT）均正常。所有发作的临床症状均完全缓解。总之，尽管初始诱发因素多样，但急性高血压似乎是儿科PRES的常见致病途径。MRI似乎优于CT，因其高分辨率和弥散加权成像而具有更高的敏感性。病变不一定位于后部白质，且可能并非完全可逆。
