AN IMMUNOLOGICAL ABNORMALITY COMMON TO BICKERSTAFFS BRAIN-STEM ENCEPHALITIS AND FISHERS SYNDROME

AN IMMUNOLOGICAL ABNORMALITY COMMON TO BICKERSTAFFS BRAIN-STEM ENCEPHALITIS AND FISHERS SYNDROME
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DOI:
10.1016/0022-510x(93)90250-3
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发表时间:
1993-08-01
影响因子:
4.4
通讯作者:
MIYATAKE, T
MIYATAKE, T
中科院分区:
医学3区
文献类型:
--
作者:
YUKI, N;SATO, S;MIYATAKE, T

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Bickerstaff's brain stem encephalitis(BBE)的疾病分类学地位尚未确定,其病因也不清楚。由于抗G(Q1b)抗体常发生在Fisher综合征(FS)患者,FS和BBE之间有临床相似性,我们调查了3例BBE患者血清中的抗神经节苷脂抗体,这些患者除了急性眼肌麻痹和小脑样共济失调外,还具有短暂的长束征,以阐明BBE的病因和疾病分类学地位。高IgG抗G(Q1b)抗体滴度存在于所有3个血清样本,但随着临床病程的疾病。与此相反,没有抗G(Q1b)抗体被发现在血清中的其他神经系统疾病,能够产生短暂的脑干障碍:多发性硬化症,神经白塞氏病,脑干梗死,单纯疱疹病毒性脑炎,韦尼克脑病。BBE和FS共享共同的自身抗体的发现表明,FS共同的自身免疫机制可能在BBE中,并且这两种情况代表了一种不同的疾病,具有广泛的症状,包括眼肌麻痹和共济失调。
The nosological position of Bickerstaff's brain stem encephalitis (BBE) has yet to be established, and its etiology is not clear. Because anti-G(Q1b) antibody frequently occurs in patients with Fisher's syndrome (FS) and there are clinical similarities between FS and BBE, we investigated anti-ganglioside antibodies in sera from 3 BBE patients who had transient long tract signs in addition to acute ophthalmoplegia and cerebellar-like ataxia in order to clarify the etiology and nosological position of BBE. High IgG anti-G(Q1b) antibody titers were present in all 3 sera samples but decreased with the clinical course of the illness. In contrast, no anti-G(Q1b) antibody was found in sera from patients with other neurologic diseases which were able to produce transient brain stem disturbance: multiple sclerosis, neuro-Behcet's disease, brain stem infarction, herpes simplex virus encephalitis, and Wernicke's encephalopathy. The finding that BBE and FS shared common autoantibody suggests that autoimmune mechanism common to FS is likely in BBE, and that both conditions represent a distinct disease with a wide spectrum of symptoms that include ophthalmoplegia and ataxia.