Department of Rheumatology, Huashan Hospital, Fudan University, Shanghai, China.
Department of Neurology, Donglei Brain Hospital, Shanghai, China.
BMC Neurol. 2022 Sep 1;22(1):329. doi: 10.1186/s12883-022-02844-6.
Acute necrotizing encephalopathy (ANE) is a rare encephalopathy characterized by multiple symmetrical brain lesions, mainly involving thalami. Adult-onset Still's disease (AOSD) is a rare systemic inflammatory condition of unknown cause characterized by fever, sore throat, rash and joint pain. Both entities are considered to be triggered by infections and associated with hypercytokinemia.
A 46-year-old male was diagnosed with AOSD at local hospital because of 3-week-long high fever, sore throat, arthralgia, transient skin rash, lymphadenopathy, leukocytosis, hyperferritinemia, and absence of antinuclear antibodies (ANA) and rheumatoid factor (RF). Corticosteroids were not used because of delayed diagnosis. Three weeks after the onset, the patient suddenly fell unconscious and was transferred to our hospital. Brain CT and MRI revealed symmetrical lesions involving thalami, striatum and brain stem, consistent with ANE. One day after admission, his condition aggravated and brain CT revealed hemorrhage in the lesions. He died 3 days after admission.
We report a rare case of ANE preceded by AOSD. The underlying mechanism is still unclear. Early recognizing of the two conditions is difficult but prognostically important.
急性坏死性脑病（ANE）是一种罕见的脑病，其特征为多灶性对称脑损伤，主要累及丘脑。成人Still 病（AOSD）是一种病因不明的罕见全身性炎症性疾病，其特征为发热、咽痛、皮疹和关节痛。这两种疾病都被认为是由感染引发的，并与细胞因子过度活跃有关。
一名 46 岁男性因 3 周高热、咽痛、关节炎、一过性皮疹、淋巴结病、白细胞增多、高铁蛋白血症和抗核抗体（ANA）及类风湿因子（RF）阴性，在当地医院被诊断为 AOSD。由于诊断延迟，未使用皮质类固醇。发病 3 周后，患者突然意识丧失并转入我院。脑 CT 和 MRI 显示双侧丘脑、纹状体和脑干对称病变，符合 ANE 表现。入院后 1 天，病情加重，脑 CT 显示病变部位出血。入院后 3 天死亡。
我们报告了一例罕见的 AOSD 后并发 ANE 的病例。其潜在机制尚不清楚。早期识别这两种疾病具有一定难度，但对预后具有重要意义。
