Zhao Mangsuo, Qiao Liyan, Shi Jie, Huang Fangjie, Zhang Mingjie, Lin Xianzhong, Wang Jing, Geng Tongchao, Zuo Huancong
Department of Neurology, Yuquan Hospital, Medical Center, Tsinghua University, No. 5 Shijingshan Road, Beijing 100049, PR China.
School of Medicine, Nankai University, Tianjin, PR China.
J Neurol Sci. 2014 Nov 15;346(1-2):227-30. doi: 10.1016/j.jns.2014.08.033. Epub 2014 Aug 28.
The cerebrospinal fluid (CSF) shows inflammatory changes in patients with idiopathic hypertrophic pachymeningitis (IHP), which is a rare disorder. However, systemic CSF research including immunoglobulins in patients with IHP are substantially lacking. In the study, clinical, laboratory, neuroradiologic and therapeutic data from 9 patients with IHP were retrospectively studied, and CSF changes were analyzed. Intracranial pressure was elevated in 4 patients. Protein levels in CSF were elevated in 5 patients (< 1g/L). IgA was elevated in 7 patients (> 0.5mg/dL), IgG was elevated in 8 patients (> 3.4 mg/dL) and IgM was elevated in 6 patients (>0.13 mg/dL) with IHP. CSF immunoglobulins, including IgA, IgG and IgM, were significantly elevated compared with levels in the control (P = 0.021, 0.018, 0.019). There were no linear correlations between IgG, IgM and protein in CSF, but there was a linear correlation between IgA and protein. In conclusion, CSF in IHP shows inflammatory changes, and protein levels are low to moderately elevated. CSF immunoglobulins, including IgA, IgG and IgM, also increased. The arachnoid is involved in IHP, a proportion of immunoglobulins may originate from the blood because of damage to the blood-CSF barrier at the arachnoid. Other intrathecal synthesis of immunoglobulins may be a secondary change due to alteration in the CSF's content to stabilize the internal environment or may be secreted by activated immune memory cells in the brain, which need further research.
特发性肥厚性硬脑膜炎（IHP）是一种罕见疾病，其患者的脑脊液（CSF）显示出炎症变化。然而，对IHP患者进行的包括免疫球蛋白在内的系统性脑脊液研究严重缺乏。在本研究中，我们对9例IHP患者的临床、实验室、神经放射学和治疗数据进行了回顾性研究，并分析了脑脊液变化。4例患者颅内压升高。5例患者脑脊液中的蛋白质水平升高（<1g/L）。IHP患者中，7例患者的IgA升高（>0.5mg/dL），8例患者的IgG升高（>3.4mg/dL），6例患者的IgM升高（>0.13mg/dL）。与对照组相比，包括IgA、IgG和IgM在内的脑脊液免疫球蛋白显著升高（P = 0.021、0.018、0.019）。脑脊液中的IgG、IgM与蛋白质之间无线性相关性，但IgA与蛋白质之间存在线性相关性。总之，IHP患者的脑脊液显示出炎症变化，蛋白质水平呈低至中度升高。包括IgA、IgG和IgM在内的脑脊液免疫球蛋白也有所增加。蛛网膜参与了IHP的发病过程，由于蛛网膜处血脑脊液屏障受损，一部分免疫球蛋白可能来源于血液。免疫球蛋白的其他鞘内合成可能是脑脊液成分改变以稳定内环境的继发变化，也可能是由脑中活化的免疫记忆细胞分泌，这需要进一步研究。
