From the Child Neurology Division (A.S., M.S., J.E.E.L., C.C., G.L.L., A.J., K.V.H.), Department of Neurology, Pediatric Neuroradiology (H.D.), Department of Radiology, Infectious Disease Division (E.A.), and Critical Care Medicine (K.G., T.J.L.), Department of Pediatrics, and Neuroimmunology Division (K.V.H.), Department of Neurology, Stanford Medicine, Palo Alto, CA.
Neurology. 2024 Oct 22;103(8):e209877. doi: 10.1212/WNL.0000000000209877. Epub 2024 Sep 19.
A previously healthy 6-year-old girl presented with several days of fever before a generalized seizure. Laboratory investigation revealed elevated liver enzymes, normal ammonia, and positive influenza A through respiratory PCR. Brain MRI demonstrated extensive, bilateral lesions in the cerebral and cerebellar white matter, thalami, basal ganglia, and brainstem. She was diagnosed with acute necrotizing encephalopathy, a rare parainfectious encephalitis commonly associated with influenza. Genetic variants have been implicated (e.g., and ), but our patient's rapid genome was nondiagnostic. Her 1-month hospitalization was complicated by prolonged encephalopathy and intracranial pressure crises requiring hyperosmolar therapy, sedation, intermittent paralysis, and hypothermia. Concomitantly, she received pulse corticosteroids, plasmapheresis, and oseltamivir. Three months after illness onset, she achieved a remarkable recovery with a normal neurologic examination. Although prognosis may comprise considerable morbidity and mortality, prompt recognition, immunotherapy, and intensive care can achieve positive neurodevelopmental outcomes. Our discussion concludes with a focus on the intrinsic uncertainties of neuroprognostication in the pediatric intensive care unit.
一位此前健康的 6 岁女孩在全身性癫痫发作前出现了数天发热。实验室检查显示肝酶升高，氨正常，呼吸道 PCR 检测甲型流感阳性。脑部 MRI 显示大脑和小脑白质、丘脑、基底节和脑干广泛的双侧病变。她被诊断为急性坏死性脑病，这是一种罕见的与流感相关的副感染性脑炎。已发现遗传变异与之相关（例如，和），但我们患者的快速全基因组检测结果没有诊断意义。她 1 个月的住院治疗过程中，因脑病持续时间长且伴有颅内压危象而变得复杂，需要高渗治疗、镇静、间歇性瘫痪和低温治疗。同时，她还接受了脉冲皮质类固醇、血浆置换和奥司他韦治疗。发病 3 个月后，她恢复良好，神经系统检查正常。尽管预后可能包括相当大的发病率和死亡率，但及时识别、免疫治疗和重症监护可以实现积极的神经发育结局。我们的讨论最后重点讨论了儿科重症监护病房中神经预后判断的内在不确定性。
