Canpolat Mehmet, Kaya Özçora Gül Demet, Poyrazoğlu Hakan, Per Huseyin, Çoşkun Abdulhakim, Gümüş Hakan, Arslan Duran, Ünal Ekrem, Karakükçü Musa, Patıroğlu Türkan, Kumandaş Sefer
Department of Pediatric Neurology, Erciyes University School of Medicine, Kayseri, Turkey.
Department of Pediatric Radiology, Erciyes University School of Medicine, Kayseri, Turkey.
Turk Arch Pediatr. 2021 Nov;56(6):569-575. doi: 10.5152/TurkArchPediatr.2021.21072.
The essential characteristics of posterior reversible encephalopathy syndrome (PRES) are the presence of acute onset neurologic symptoms, focal vasogenic edema at neuroimaging, and reversible clinical and/or radiologic findings. This study aimed to evaluate the clinical findings, causes, radiologic findings, and prognoses of patients with PRES.
Patients with PRES confirmed with clinical and radiologic findings by a pediatric neurologist were evaluated retrospectively.
Seventeen patients with PRES were evaluated (mean age at onset, 10.23 ± 4.65 years; range, 2-17 years; girls, 29.4% [n = 5]). The mean length of follow-up was 6 ± 2.3 years (range, 3.4-10 years). Mortality due to primary disease occurred in 4 patients (23.5%) during follow-up. PRES was derived from renal diseases in 10 patients (58.8%), hematologic diseases in 6 patients (35.3%), and liver disease in one patient (5.9%). Hypertension was present in 16 patients (94.1%) at onset of PRES (>99th percentile). Seizure, the most frequent initial symptom, was observed in 82.4% (n = 14). Blurred vision and headache were the initial symptoms in 3 patients (17.6%). Sequelae were observed at magnetic resonance imaging (MRI) in 6 patients. Development of epilepsy was determined as a sequela in 4 patients (23.5%) and mental motor retardation in 2 patients (11.8%).
Epilepsy is uncommon in patients who have recovered from PRES. The presence of gliosis on MRI and interictal epileptic discharges on electroencephalograms are major risk factors for the development of epilepsy. Antiepileptic treatment can be stopped in the early period in patients with normal MRI and electroencephalogram by eliminating the factors that trigger the seizures.
后部可逆性脑病综合征(PRES)的基本特征是急性起病的神经系统症状、神经影像学上的局灶性血管源性水肿以及可逆的临床和/或影像学表现。本研究旨在评估PRES患者的临床表现、病因、影像学表现及预后。
对经儿科神经科医生根据临床和影像学检查确诊为PRES的患者进行回顾性评估。
共评估了17例PRES患者(发病时平均年龄为10.23±4.65岁;范围为2 - 17岁;女孩占29.4%[n = 5])。平均随访时间为6±2.3年(范围为3.4 - 10年)。随访期间有4例患者(23.5%)因原发性疾病死亡。10例患者(58.8%)的PRES源于肾脏疾病,6例患者(35.3%)源于血液系统疾病,1例患者(5.9%)源于肝脏疾病。PRES发病时16例患者(94.1%)存在高血压(>第99百分位数)。癫痫是最常见的首发症状,82.4%(n = 14)的患者出现该症状。3例患者(17.6%)的首发症状为视力模糊和头痛。6例患者在磁共振成像(MRI)检查时出现后遗症。4例患者(23.5%)被确定有癫痫后遗症,2例患者(11.8%)有精神运动发育迟缓后遗症。
从PRES恢复的患者中癫痫并不常见。MRI上出现胶质增生以及脑电图上出现发作间期癫痫样放电是癫痫发生的主要危险因素。对于MRI和脑电图正常的患者,通过消除引发癫痫发作的因素,可在早期停用抗癫痫治疗。