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[大剂量静脉注射免疫球蛋白治疗儿童Bickerstaff脑干脑炎一例]

[High-dose intravenous immunoglobulin therapy in a child case of Bickerstaff's brainstem encephalitis].

作者信息

Sugiyama Nobuyoshi, Hamano Shin-ichiro, Koga Michiaki

机构信息

Division of Neurology, Saitama Children's Medical Center, Iwatsuki, Saitama.

出版信息

No To Hattatsu. 2003 Jul;35(4):327-30.

Abstract

We report an 11-year-old boy with Bickerstaff's brainstem encephalitis (BBE). He had gait disturbance, disturbed consciousness, and diplopia after upper respiratory tract infection. On admission, he showed multiple cranial nerve palsy, muscle weakness of arms, cerebeller ataxia and generalized areflexia. The cerebrospinal fluid on day 7 revealed albuminocytologic dissociation. IgG antibodies against GQ1b and GT1a were detected in the serum. Immunoglobulin was administered intravenously from day 11, and then his symptoms gradually diminished. When he was discharged on day 27, he had neither conscious disturbance nor limb weakness. There still were mild ophthalmoparesis and diminished deep tendon reflexes, but they disappeared by 10 months after the onset. Effective therapy for BBE has yet to be established. Our case had features of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome, such as an acute monophasic course, limb weakness with areflexia, albuminocytologic dissociation in the cerebrospinal fluid, detection of serum anti-ganglioside antibodies and efficacy of intravenous immunoglobulin, indicating that BBE and GBS are closely related. Our case suggested that intravenous immunoglobulin therapy, an established treatment for GBS, should be considered in some patients with BBE.

摘要

我们报告了一名患有比克斯特法脑干脑炎(BBE)的11岁男孩。他在上呼吸道感染后出现步态障碍、意识障碍和复视。入院时,他表现出多组颅神经麻痹、手臂肌肉无力、小脑共济失调和全身反射消失。第7天的脑脊液显示蛋白细胞分离。血清中检测到抗GQ1b和GT1a的IgG抗体。从第11天开始静脉注射免疫球蛋白,随后他的症状逐渐减轻。第27天出院时,他既没有意识障碍也没有肢体无力。仍有轻度眼肌麻痹和腱反射减弱,但在发病后10个月时消失。BBE的有效治疗方法尚未确立。我们的病例具有吉兰 - 巴雷综合征(GBS)和米勒 - 费希尔综合征的特征,如急性单相病程、肢体无力伴反射消失、脑脊液蛋白细胞分离、血清抗神经节苷脂抗体检测以及静脉注射免疫球蛋白的疗效,表明BBE与GBS密切相关。我们的病例提示,对于一些BBE患者,应考虑使用已确立的GBS治疗方法——静脉注射免疫球蛋白治疗。

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