Akiyoshi Kensuke, Hamada Yumi, Yamada Hiroshi, Kojo Masanobu, Izumi Tatsuro
Division of Pediatrics and Child Neurology, Department of Brain and Nerve Science, Oita University Faculty of Medicine, Hasama, Oita, Japan.
Pediatr Neurol. 2006 Apr;34(4):315-8. doi: 10.1016/j.pediatrneurol.2005.08.030.
A 7-year-old female suddenly exhibited high fever and convulsions, and entered a semi-coma. She also had thrombocytopenia, elevated aminotransferase, prolonged prothrombin time and activated partial thromboplastin time, and hemophagocytes in the bone marrow. The brain magnetic resonance imaging revealed multiple low-intensity areas on the T1-weighted images, and high-intensity areas on the T2-weighted images bilaterally in the thalamus, the dorsal part of the pons, and the cerebellar white matter. The patient was diagnosed as having both acute necrotizing encephalopathy and hemophagocytic syndrome. Serum and cerebrospinal fluid interleukin-6 and tumor necrosis factor-alpha were elevated to the same high levels (serum:cerebrospinal fluid interleukin-6, 103:101 pg/mL; tumor necrosis factor-alpha 753:753 pg/mL). The clinical symptoms and the magnetic resonance imaging findings improved immediately after the administration of dexamethasone. These results suggest that the hypercytokinemia and the hyperpermeability of both the blood-brain barrier and the capillary walls of the central nervous system might be essential in the pathogenesis of acute necrotizing encephalopathy, and that early steroid therapy might be effective in these conditions.
一名7岁女性突然出现高热和惊厥，并进入半昏迷状态。她还出现血小板减少、转氨酶升高、凝血酶原时间和活化部分凝血活酶时间延长，骨髓中可见噬血细胞。脑部磁共振成像显示，在T1加权图像上有多个低强度区域，在丘脑、脑桥背侧和小脑白质双侧的T2加权图像上有高强度区域。该患者被诊断为患有急性坏死性脑病和噬血细胞综合征。血清和脑脊液中的白细胞介素-6和肿瘤坏死因子-α升高至相同的高水平（血清：脑脊液白细胞介素-6，103:101 pg/mL；肿瘤坏死因子-α 753:753 pg/mL）。给予地塞米松后，临床症状和磁共振成像结果立即改善。这些结果表明，高细胞因子血症以及血脑屏障和中枢神经系统毛细血管壁的高通透性可能在急性坏死性脑病的发病机制中起重要作用，并且早期类固醇治疗在这些情况下可能有效。
