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目的 探讨副肿瘤性脑脊髓炎 /感觉神经元病 (PEM/ PSN)的发病机制。方法 采用亲和免疫细胞组织化学 (ABC)法测定 16例 PEM及 14例 PSN患者血清及脑脊液 (CSF)中的抗核抗体 (抗 Hu抗体 )的滴度 ,将所得的血清及 CSF抗 Hu抗体滴度按照 Schuller数学模拟公式计算出鞘内抗 Hu抗体的合成及抗 Hu抗体的特异活性 (ASA) ;采用免疫印迹分析 ,测定抗 Hu抗体所识别的大脑神经元胞核提取物中蛋白抗原 ;采用火箭免疫电泳测定血清、CSF中的 Ig G以及 CSF中的白蛋白含量。结果 PEM患者 CSF中的抗 Hu抗体滴度高于 PSN患者 (P<0 .0 0 1)。 16例 PEM中有 14例 (88% ) ,而 14例 PSN中仅有 1例 (7% ) CSF ASA/血清 ASA比值 >2。表明 14例PEM和 1例 PSN患者存在鞘内抗 Hu抗体的合成 ,两组间差异有显著性意义 (P<0 .0 0 1)。 PEM患者神经功能损害严重 ,而 PSN患者神经功能损伤较轻。两组 Rankin等级评分值分别为 (4.5± 0 .7)和 (3.0± 1.4)分 ,两组相比差异有显著性 (P<0 .0 1)。结论 检测鞘内 ASA/血清 ASA比值 ,验证鞘内是否有抗 Hu抗体合成 ,对于判断神经系统副肿瘤综合征疾病严重程度及预后有一定意义
Objective To investigate the pathogenesis of paraneoplastic encephalomyelitis/sensory neuron disease (PEM/PSN). Methods The titer of anti-nuclear antibody (anti-Hu antibody) in serum and cerebrospinal fluid (CSF) of 16 patients with PEM and 14 patients with PSN was measured by affinity immunohistochemistry (ABC) method. The serum and CSF anti-Hu antibody were obtained. The titer was calculated according to Schuller’s mathematical simulation formula and the anti-Hu antibody synthesis and anti-Hu antibody specific activity (ASA) was calculated. Western blot analysis was used to determine protein antigens in the nuclear extracts of brain neurons identified by anti-Hu antibodies; Determination of serum albumin, Ig G in CSF, and albumin in CSF were performed using rocket immunoelectrophoresis. Results The anti-Hu antibody titer in CSF was higher in PEM patients than in PSN patients (P < 0.01). Of the 16 patients with PEM, 14 (88%), and only 1 (14%) of the 14 PNS had CSF ASA/serum ASA ratio >2. The results showed that 14 cases of PEM and 1 case of PSN had intrathecal anti-Hu antibody synthesis, and the difference between the two groups was significant (P < 0.01). PEM patients with severe neurological impairment, and PSN patients with less neurological damage. The Rankin scores of the two groups were (4.5 ± 0.7) and (3.0 ± 1.4) points, respectively. There was a significant difference between the two groups (P < 0.01). Conclusion The detection of intrathecal ASA/serum ASA ratio to verify the presence of anti-Hu antibody synthesis in the sheath is of significance in judging the severity and prognosis of paraneoplastic syndromes in the nervous system.