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一名接受胸腺切除术治疗的重症肌无力患者出现无症状性脑脊液寡克隆IgG及鞘内IgG合成逐渐增加——4年随访

Asymptomatic oligoclonal CSF IgG and progressive increase of intrathecal IgG synthesis in a patient with myasthenia gravis treated with thymectomy--a 4-years follow-up.

作者信息

Kam-Hansen S

出版信息

J Neurol Sci. 1986 Oct;75(3):285-92. doi: 10.1016/0022-510x(86)90076-6.

Abstract

A young woman is described who had myasthenia gravis which was favourably influenced by thymectomy. Although there was no evidence for another neurological disease over 7.5 years of clinical observation and computed tomography as well as visual evoked response were normal, CSF studies over 0.5 years prior to and 3.5 years after thymectomy revealed persistent mononuclear pleocytosis, high CSF IgG index, oligoclonal IgG bands in CSF, and increasing IgG synthesis rate within the CNS, reflecting a continuous local humoral immune response. CSF/serum ratios of antibodies to acetylcholine receptors (AChR) were continuously lower than CSF/serum IgG ratios, contradicting intrathecal AChR antibody production. Proportion of total T cells was slightly higher in CSF than peripheral blood, while active T cells were lower in CSF. CSF lymphocytes did not proliferate on PHA stimulation but responded in allogeneic mixed lymphocyte culture. B cells were 4% in CSF and 4.5% in peripheral blood, but 227 IgG, 0 IgA and 0 IgM producing cells were detected among 20 X 10(3) CSF lymphocytes, compared to 5, 4 and 0 in 20 X 10(3) peripheral blood lymphocytes. This patient represents an in vivo 'experiment' regarding influence of thymectomy on CSF compartment constituents. The present study also shows that an individual can be clinically healthy despite continuous and pronounced intrathecal immune response.

摘要

本文描述了一名患有重症肌无力的年轻女性,胸腺切除术对其病情产生了积极影响。尽管在7.5年的临床观察中未发现其他神经系统疾病的证据,计算机断层扫描和视觉诱发电位均正常,但在胸腺切除术前0.5年和术后3.5年的脑脊液研究显示,持续存在单核细胞增多、脑脊液IgG指数升高、脑脊液中寡克隆IgG带以及中枢神经系统内IgG合成率增加,反映了持续的局部体液免疫反应。脑脊液/血清中乙酰胆碱受体(AChR)抗体的比率持续低于脑脊液/血清IgG比率,这与鞘内AChR抗体产生相矛盾。脑脊液中总T细胞的比例略高于外周血,而活化T细胞在脑脊液中较低。脑脊液淋巴细胞在PHA刺激下不增殖,但在同种异体混合淋巴细胞培养中反应。脑脊液中B细胞占4%,外周血中占4.5%,但在20×10³个脑脊液淋巴细胞中检测到227个IgG产生细胞、0个IgA产生细胞和0个IgM产生细胞,而在20×10³个外周血淋巴细胞中分别为5个、4个和0个。该患者代表了胸腺切除术对脑脊液成分影响的体内“实验”。本研究还表明,尽管存在持续且明显的鞘内免疫反应,个体在临床上仍可能健康。

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