PDF(Pubmed)
Abstract：
A 4-year-old boy with Nuclear factor-kappa B Essential Modulator deficiency syndrome presented with encephalopathy post haematopoietic stem cell transplantation. MRI demonstrated T2/FLAIR-hyperintensities in the posterior cerebral cortex concerning for posterior reversible encephalopathy syndrome. Clinical improvement was appreciated following withdrawal of the suspected offending pharmacological agent (Cyclosporine). An 18F-FDG PET/CT performed 2 months later to screen for post-transplant lymphoproliferative disease demonstrated markedly reduced FDG uptake in the posterior cerebral cortex, involving the parietal and occipital lobes. We describe, to the best of our knowledge, the first case of profound cerebral hypometabolism in a child with clinically resolved posterior reversible encephalopathy syndrome.
摘要：
一名4岁男孩，患有核因子-κB必需调节剂缺乏综合征，在造血干细胞移植后出现脑病。MRI显示后大脑皮层T2/FLAIR高信号，涉及后部可逆性脑病综合征。在停用可疑的药理药物（环孢菌素）后，临床改善得到了赞赏。2个月后进行的18F-FDGPET/CT筛查移植后淋巴增殖性疾病显示，后大脑皮层中FDG的摄取显着降低，涉及顶叶和枕叶。我们描述，据我们所知,首例患有临床解决的可逆性后部脑病综合征的儿童发生深度脑代谢不足。
