From the Departments of Pediatrics (J.G., R.C., P.S., R.N.S., K.D.), and Radio-Diagnosis (A.G.), SMS Medical College; and SK Babylon's Newton Institute of Child and Adolescent Development (S.S.), Jaipur, India.
Neurology. 2024 Nov 12;103(9):e209954. doi: 10.1212/WNL.0000000000209954. Epub 2024 Oct 7.
Acute necrotizing encephalopathy of childhood (ANEC) is characterized by a rapidly progressive encephalopathy after a viral febrile illness, with multiple, symmetrical lesions in the brain including the thalami. Acute dengue fever is a known, but rare, trigger for ANEC. The clinical course of ANEC is usually fulminant and is associated with high morbidity and mortality. We describe here a prospective cohort of 5 children who presented with ANEC associated with dengue infection characterized by encephalopathy within the first week of fever, followed by a rapidly deteriorating sensorium requiring intensive care unit admission. Dengue fever was diagnosed based on a positive nonstructural protein 1 antigen test. ANEC was diagnosed based on characteristic MRI brain findings including the "trilaminar sign" in bilateral thalami combined with clinical features of severe encephalopathy, thrombocytopenia, and transaminitis. All the children required prolonged hospital stay (mean duration 30 days), with 4 of 5 children requiring mechanical ventilation (mean duration 21 days). All the children received immunomodulation with IV methylprednisolone either alone or followed by IV immunoglobulin. Although these children were bedridden at discharge (modified Rankin Scale, mRS score 4 or 5), they showed a consistent recovery in follow-up. At 6 months of follow-up, all the children were fully independent in activities of daily living (mRS scores 1-2). These cases highlight good neurologic outcomes in children with ANEC associated with dengue despite a catastrophic presentation and a protracted hospital course. The most common residual neurologic deficits noted were hand tremors and extrapyramidal dysarthria.
儿童急性坏死性脑病 (ANEC) 的特征是在病毒发热性疾病后迅速进展的脑病，伴有大脑包括丘脑在内的多发性、对称性病变。急性登革热是已知的但罕见的 ANEC 触发因素。ANEC 的临床病程通常是暴发性的，与高发病率和死亡率相关。我们在此描述了一组 5 例儿童前瞻性队列研究，这些儿童患有与登革热感染相关的 ANEC，其特征是在发热的第一周内出现脑病，随后意识迅速恶化，需要入住重症监护病房。登革热是基于非结构蛋白 1 抗原检测阳性来诊断的。ANEC 是基于特征性 MRI 脑发现来诊断的，包括双侧丘脑的“三层征”，并结合严重脑病、血小板减少和转氨酶升高的临床特征。所有儿童都需要长时间住院（平均住院时间 30 天），其中 5 名儿童中有 4 名需要机械通气（平均持续时间 21 天）。所有儿童都接受了免疫调节治疗，单独使用 IV 甲基强的松龙或随后使用 IV 免疫球蛋白。尽管这些儿童在出院时（改良 Rankin 量表，mRS 评分 4 或 5）卧床不起，但在随访中显示出一致的恢复。在 6 个月的随访中，所有儿童在日常生活活动中完全独立（mRS 评分 1-2）。这些病例突出表明，尽管表现为灾难性且住院时间延长，但与登革热相关的 ANEC 儿童仍有良好的神经预后。注意到的最常见的残留神经缺陷是手部震颤和锥体外系构音障碍。
