Department of Pediatrics, University Hospital of Patras, Greece.
Pediatr Neurol. 2013 Sep;49(3):213-7. doi: 10.1016/j.pediatrneurol.2013.04.024. Epub 2013 Jul 4.
Children with sickle cell disease are at increased risk for neurological complications. Reversible posterior leukoencephalopathy syndrome is a clinico-radiological syndrome characterized by headache, vomiting, seizures, and visual disturbances along with the radiological findings of posterior leukoencephalopathy.
We report a 13-year-old female with sickle cell disease presenting with a headache and two isolated episodes of partial seizures with secondary generalization after blood transfusion. The brain MRI revealed bilateral high signal areas on T2 images in parietal, occipital, and frontal lobes; in the cerebellum; midbrain; and within the right caudate nucleus. Follow-up MRI 2 weeks later showed almost complete resolution of the previously detected findings. The clinical presentation along with the reversible aforementioned neuroimaging findings indicated reversible posterior leukoencephalopathy syndrome as the most appropriate diagnosis. Although the syndrome has been described previously in various clinical conditions, this is the first time that it is recognized after blood transfusion in an adolescent with sickle cell disease.
Neurological deterioration in children with sickle cell disease after blood transfusion should raise high suspicion for reversible posterior leukoencephalopathy syndrome, especially in the setting of elevated blood pressure.
镰状细胞病患儿存在神经并发症风险增加的情况。可逆性后部白质脑病综合征是一种临床-放射学综合征，其特征为头痛、呕吐、癫痫发作和视觉障碍，同时伴有后部白质脑病的放射学表现。
我们报告了一例 13 岁镰状细胞病女性患者，在输血后出现头痛和两次孤立性部分性癫痫发作，随后出现全身性发作。脑部 MRI 显示双侧顶叶、枕叶和额叶、小脑、中脑和右侧尾状核的 T2 图像上有高信号区。2 周后的随访 MRI 显示之前发现的病变几乎完全消退。临床表现以及上述可逆转的神经影像学发现表明，最适合的诊断是可逆性后部白质脑病综合征。尽管该综合征以前在各种临床情况下均有描述，但这是首次在镰状细胞病青少年输血后发现。
镰状细胞病患儿输血后出现神经恶化应高度怀疑可逆性后部白质脑病综合征，尤其是在血压升高的情况下。
