Yae Yukako, Kawano Go, Yokochi Takaoki, Imagi Toru, Akita Yukihiro, Ohbu Keizo, Matsuishi Toyojiro
Department of Pediatrics, St Mary's Hospital, 422 Tsubukuhonmachi, Kurume, Fukuoka 830-8543, Japan.
Department of Pediatrics, St Mary's Hospital, 422 Tsubukuhonmachi, Kurume, Fukuoka 830-8543, Japan; Research Centre for Children and Research Centre for Rett Syndrome, St Mary's Hospital, 422 Tsubukuhonmachi, Kurume, Fukuoka 830-8543, Japan.
Brain Dev. 2019 Apr;41(4):373-377. doi: 10.1016/j.braindev.2018.11.007. Epub 2018 Dec 3.
Acute disseminated encephalomyelitis (ADEM) is a typically monophasic inflammatory demyelinating disease of the central nervous system with a favorable outcome. However, 2% of ADEM involves acute hemorrhagic leukoencephalitis (AHLE), which is a fulminant and hyperacute variant of ADEM with a poor outcome and high mortality. There are limited case reports of fulminant ADEM including AHLE in children. Herein, we report two pediatric cases of fulminant ADEM. Both cases had a rapid deterioration of consciousness, repetitive seizures, and brain edema on neuroimaging, in addition to atypical neuroradiological findings on magnetic resonance imaging (MRI), a reversible splenial lesion in case 1, and bilateral frontal and occipital cortical lesions in case 2. Both cases were treated with early high-dose methyl-prednisolone and immunoglobulin, while therapeutic hypothermia was also initiated in case 2 after the patient exhibited a decerebrate posture and irregular breathing pattern. Both cases had a favorable outcome. Further case reports on pediatric fulminant ADEM are required to clarify the various clinical types, and to examine the efficacy of various treatment modalities for fulminant ADEM and AHLE in children.
急性播散性脑脊髓炎（ADEM）是一种典型的中枢神经系统单相性炎性脱髓鞘疾病，预后良好。然而，2%的ADEM会发展为急性出血性白质脑炎（AHLE），这是ADEM的一种暴发性和超急性变体，预后不良且死亡率高。儿童暴发性ADEM（包括AHLE）的病例报告有限。在此，我们报告两例儿童暴发性ADEM病例。两例病例均出现意识迅速恶化、反复癫痫发作以及神经影像学检查显示脑水肿，此外，磁共振成像（MRI）还发现了非典型神经放射学表现，病例1为可逆性胼胝体病变，病例2为双侧额叶和枕叶皮质病变。两例病例均早期接受了大剂量甲基强的松龙和免疫球蛋白治疗，病例2在出现去大脑强直姿势和不规则呼吸模式后还启动了亚低温治疗。两例病例均预后良好。需要更多儿童暴发性ADEM的病例报告来明确各种临床类型，并研究针对儿童暴发性ADEM和AHLE的各种治疗方式的疗效。
