Akhondian Javad, Ashrafzadeh Farah, Seilanian Toosi Farrokh, Behnam Mahdi, Beiraghi Toosi Mehran, Imannezhad Shima, Akhoundian Mohammad Reza, Hashemi Narges
Department of Pediatrics , School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.
Department of Radiology, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.
Iran J Child Neurol. 2022 Spring;16(2):149-154. doi: 10.22037/ijcn.v16i1.32170. Epub 2022 Mar 14.
Posterior reversible encephalopathy syndrome (PRES) has a broad spectrum of clinical presentations and radiological features. Diagnosis of PRES is established based on reversible clinical manifestations and sequential neuroimaging findings. Atypical MRI features include hemorrhage, restricted diffusion or contrast enhancement of lesions, and involvement of the temporal and frontal lobes, brainstem, basal ganglia, corpus callosum, cerebellum, and spine. Atypical PRES, with or without spinal cord involvement, is a rare presentation, especially in children. Until 2020, only five cases of PRES with spinal cord involvement (PRES-SCI) were reported in the pediatric population.
Here, we present the youngest diagnosed case of PRES-SCI so far. According to the literature, all six cases of PRES-SCI showed high signal intensities on T2-weighted images of the brainstem and cervical cord, which had completely resolved in the follow-up MRI of the brain and spinal cord. All six patients had hypertension due to renal disease, except one girl with chemotherapy-induced hypertension. Headache, altered mental status, seizure, and visual impairment were the most common symptoms, respectively. Facial palsy was a remarkable warning sign in some patients before hospitalization.Although PRES-SCI is rare in children, since it is a reversible condition, prompt diagnosis and management can positively affect its prognosis.
后部可逆性脑病综合征(PRES)具有广泛的临床表现和影像学特征。PRES的诊断基于可逆的临床表现和连续的神经影像学检查结果。非典型MRI特征包括出血、病变的扩散受限或强化以及颞叶、额叶、脑干、基底节、胼胝体、小脑和脊柱受累。伴有或不伴有脊髓受累的非典型PRES是一种罕见的表现,尤其是在儿童中。截至2020年,儿科人群中仅报告了5例伴有脊髓受累的PRES(PRES-SCI)。
在此,我们展示了迄今为止诊断出的最年轻的PRES-SCI病例。根据文献,所有6例PRES-SCI在脑干和颈髓的T2加权图像上均显示高信号强度,在脑部和脊髓的随访MRI中已完全消失。除1名因化疗引起高血压的女孩外,所有6例患者均因肾病患有高血压。头痛、精神状态改变、癫痫发作和视力障碍分别是最常见的症状。面神经麻痹在一些患者住院前是一个显著的警示信号。尽管PRES-SCI在儿童中罕见,但由于它是一种可逆性疾病,及时诊断和治疗可对其预后产生积极影响。