急性坏死性脑病的诊断与鉴别诊断

Diagnosis and differential diagnosis of acute necrotizing encephalopathy in children

目的探讨急性坏死性脑病（acute necrotizing encephalopathy, ANE）的诊断与治疗，降低其病死率。方法对4例经治的ANE患儿的临床资料进行回顾性分析。结果男2例，女2例。年龄分别为4、5、15、22个月。主要临床特点为：（1）急性起病；（2）均有发热。体温在38～40℃之间；（3）多在发病的24～48h内迅速出现昏迷；（4）均有惊厥发作，表现为全面性或局灶性发作，2例患儿呈惊厥持续状态；（5）3例重者呈去皮层状态；（6）头部MRI除双侧丘脑对称性长T1、长T2信号外。大脑及小脑髓质、脑干也有受累；（7）血清GPT、CPK有不同程度升高，血氨多为正常或轻度升高，血糖正常；（8）脑脊液。除1例轻度升高外。其他均正常；脑脊液病毒学、细菌学及肺炎支原体检查阴性；（9）脑电图在广泛性慢波背景下，可显示棘慢波灶；（10）预后差。其中1例死亡。1例呈植物状态，1例留有癫痫，1例正常。结论ANE无确切诊断指标，通常根据临床资料进行综合判定，因此，鉴别诊断十分重要。

Objective To investigate the diagnosis and treatment of acute necrotizing encephalopathy （ANE） in order to reduce its mortality. Methods The clinical profiles collected from 4 children with ANE were analyzed respectively. Results Two males and 2 females were included with age ranged from 4 to 22 months. In our cases, ANE were clinically characterized in all cases by abrupt onset, high fever with temperature about 38- 40 ℃, coma which quickly occurred within 24 - 48 h from onset of the illness, convulsion manifested as generalized or focal seizure with 2 cases as epileptica status, and limbs spasm with decortical rigidity in 3 severe cases. MRI of the brain showed symmetrical thalamic lesions, and cerebrum, cerebellum and brainstem were also involved. Serum CPK and GPT were elevated to different degree. Serum ammonium level was normal or slightly elevated. Blood sugar was normal in all the cases. Examination of CSF was normal except that protein content was high in one case. EEG showed slow wave activity with spike-slow waves. The prognosis of ANE was not satifactory. Of the 4 cases, one died, one was in vegetative state, one sustained epilepsy, and one was merely normal. Conclusion The mechanism of ANE remains unclear, which is assumed to be mediated through cytokines induced by viruses, or mycoplasma pneumoniae infection. Differential diagnosis is very important since there are no exact diagnostic criteria for ANE.