Largo Prisca, Arnone Olivia C, Sacco Francesco, Cantalupo Gaetano, Biban Paolo
Pediatric Intensive Care Unit, University Hospital of Verona, Piazzale Stefani 1, 37126 Verona, Italy.
School of Pediatrics, University of Verona, 37126 Verona, Italy.
Reports (MDPI). 2024 Dec 22;7(4):118. doi: 10.3390/reports7040118.
: Acute necrotizing encephalopathy (ANE) represents a severe complication, mainly described in children, of influenza virus infection. We report the cases of two young girls with ANE associated with influenza virus infection who were diagnosed by MRI cerebral scan. : A 7-year-old girl with a history of a previous episode of ANE presented with a worsening drowsy state and seizures. In the second case, an otherwise healthy 5-year-old girl presented with fever, seizures, and marked neurological deterioration. In both cases, nasopharyngeal swab testing was positive for influenza virus A, while cerebral MRI indicated ANE. Despite aggressive treatment with high-dose corticosteroids and a five-day course ofimmunoglobulins, the ultimate prognosis was poor in both patients. ANE is a serious complication of viral infections in children, with a high mortality rate and a broad spectrum of neurological sequelae. To date, the pathophysiology and management of influenza virus-induced ANE remain uncertain. Although ANE is usually sporadic, familial and recurrent cases have been reported, and anRAN-binding protein (RANBP2) mutation has occasionally been associated with its occurrence.Conclusions: Rapid recognition of neurological symptoms and suspicion of a viral trigger, especially in influenza-like illnesses, are both essential for the timely administration of effective therapy. Further research is needed to clarify the pathophysiology of ANE and establish the best therapeutic strategies to fight such a deadly disease.
急性坏死性脑病(ANE)是流感病毒感染的一种严重并发症,主要见于儿童。我们报告两例经MRI脑部扫描诊断为与流感病毒感染相关的ANE的年轻女孩病例。一名有ANE既往发作史的7岁女孩出现嗜睡状态恶化和癫痫发作。在第二例中,一名原本健康的5岁女孩出现发热、癫痫发作和明显的神经功能恶化。两例患者鼻咽拭子检测甲型流感病毒均呈阳性,而脑部MRI显示为ANE。尽管采用大剂量皮质类固醇积极治疗并进行了为期五天的免疫球蛋白治疗,但两名患者的最终预后均较差。ANE是儿童病毒感染的一种严重并发症,死亡率高,神经后遗症范围广泛。迄今为止,流感病毒诱导的ANE的病理生理学和治疗方法仍不确定。虽然ANE通常为散发性,但也有家族性和复发病例的报道,且偶尔与RAN结合蛋白(RANBP2)突变有关。结论:快速识别神经症状并怀疑病毒触发因素,尤其是在流感样疾病中,对于及时给予有效治疗至关重要。需要进一步研究以阐明ANE的病理生理学并确立对抗这种致命疾病的最佳治疗策略。