儿童急性坏死性脑病诊疗进展

Advances in the diagnosis and treatment of acute necrotizing encephalopathy of childhood

儿童急性坏死性脑病(acute necrotizing encephalopathy of childhood,ANEC)是一种儿童期罕见发生继发感染的急性脑病,伴随发热、惊厥、意识障碍甚至昏迷,病程进展迅速,发病机制可能与感染后体内免疫反应过激相关;病理学改变为血管损伤引起渗透压改变,致血脑屏障破坏,血浆渗出,引起脑组织水肿、点状出血、神经元及胶质细胞坏死;影像学表现较典型,可呈现对称的多灶性损伤,多累及双侧丘脑等;ANEC尚无特异性治疗,预后多伴有不同程度的神经后遗症甚至死亡。

Acute necrotizing encephalopathy of childhood(ANEC)is a rare acute encephalopathy with secondary infection in childhood,accompanied by fever,convulsion,disturbance of consciousness and even coma.The course of the disease progresses rapidly.The pathogenesis may be related to excessive immune response after infection.The pathological changes are as follows:vascular injury leading to the changes of osmotic pressure and destruction of blood-brain barrier,plasma exudation,brain edema,punctate hemorrhage,neuron and glial cell necrosis.Imaging findings are typical and show symmetrical multifocal injury,involving bilateral thalamus and so on.There is no specific treatment for ANEC,and most of the prognosis is accompanied by different degrees of neurological sequelaes even death.