Ma Juan, Pan Chenliang, Bai Nan, Zhang Shanshan, Mi Peiling, Wa Yongling, Lu Andong
Department of Cardiovascular Medicine, The First Hospital of Lanzhou University, Lanzhou City, Gansu, China.
Front Cardiovasc Med. 2025 May 27;12:1574397. doi: 10.3389/fcvm.2025.1574397. eCollection 2025.
Acute necrotizing encephalopathy (ANE) is a rare condition characterized by multiple symmetrical brain lesions mainly involving the thalamus. Acute fulminant myocarditis is a diffuse inflammatory disease of the myocardium characterized by acute onset, rapid progression, and a high risk of death. Its pathogenesis involves excessive activation of the innate immune system and the formation of an inflammatory storm. Both conditions are thought to be caused by viral infections. We present a case of ANE with fulminant myocarditis. Reporting this case is important due to the rarity and the critical interplay of these two severe conditions occurring simultaneously.
A 16-year-old student presented with a 3-day history of high fever, cough, and expectoration, followed by multiple episodes of convulsive seizures. Despite high doses of vasoactive medications, the patient exhibited low blood pressure and elevated lactate levels. Portable echocardiography revealed diffuse decreased left ventricular motion with severe left ventricular dysfunction (ejection fraction < 30% by visual estimation). The patient was diagnosed with acute fulminant myocarditis. The patient remained comatose with a Glasgow coma scale (GCS) score of 3 (E1VeM1). Brain CT and MRI revealed bilateral striatal, thalamic, and brainstem lesions, typical of ANE. Consequently, a diagnosis of ANE accompanied by fulminant myocarditis was considered. The treatment regimen included high doses of glucocorticoids, immunoglobulins, tocilizumab, and V-A ECMO (Veno-arterial extracorporeal membrane oxygenation) life support. The patient showed significant recovery of cardiac function and was discharged after approximately 24 days of rehabilitation.
This case report highlights the coexistence of ANE and fulminant myocarditis. The underlying mechanisms remain unclear. Early recognition of these two conditions is crucial for prognosis, though challenging. This report underscores the need for heightened awareness and prompt, comprehensive treatment strategies to improve outcomes in such complex cases.
急性坏死性脑病（ANE）是一种罕见疾病，其特征为主要累及丘脑的多发性对称性脑病变。急性暴发性心肌炎是一种心肌弥漫性炎症性疾病，其特点是起病急、进展快、死亡风险高。其发病机制涉及先天性免疫系统的过度激活和炎症风暴的形成。这两种疾病都被认为是由病毒感染引起的。我们报告一例伴有暴发性心肌炎的ANE病例。由于这两种严重疾病同时发生的罕见性以及关键的相互作用，报告该病例具有重要意义。
一名16岁学生出现高热、咳嗽和咳痰3天病史，随后多次发生惊厥性癫痫发作。尽管使用了高剂量血管活性药物，患者仍出现低血压和乳酸水平升高。便携式超声心动图显示左心室弥漫性运动减弱，左心室功能严重受损（目测射血分数<30%）。该患者被诊断为急性暴发性心肌炎。患者一直昏迷，格拉斯哥昏迷量表（GCS）评分为3分（E1VeM1）。脑部CT和MRI显示双侧纹状体、丘脑和脑干病变，为ANE的典型表现。因此，考虑诊断为ANE伴暴发性心肌炎。治疗方案包括高剂量糖皮质激素、免疫球蛋白、托珠单抗和静脉-动脉体外膜肺氧合（V-A ECMO）生命支持。患者心脏功能显著恢复，康复约24天后出院。
本病例报告突出了ANE和暴发性心肌炎的共存。其潜在机制仍不清楚。尽早识别这两种疾病对预后至关重要，尽管具有挑战性。本报告强调需要提高认识，并采取及时、全面的治疗策略，以改善此类复杂病例的治疗效果。
