Comparison of cerebrospinal fluid characteristics in three rare types of autoimmune inflammatory demyelinating diseases

doi:10.3969/j.issn.1672-6731.2025.12.005

Abstract

Abstract:

Objective: To explore the cerebrospinal fluid (CSF) results of anti-aquaporin-4 (AQP4) IgG positive neuromyelitis optica spectrum disorders (NMOSDs), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) and compare their similarities and differences to provide a reference for its diagnosis and differential diagnosis. Methods and Results: From April 2019 to August 2023, 38 patients with serum AQP4 -IgG-positive for NMOSDs, 14 patients with MOGAD and 27 patients with GFAP-A who were hospitalised in General Hospital of Ningxia Medical University were selected. The clinical data and CSF pressure, cytology, biochemistry and IgG of the 3 groups were collected after admission. Compared with the NMOSDs group, the MOGAD group and the GFAP -A group, the CSF pressure (F = 10.530, P = 0.000), white blood cell (WBC) count (χ² = 11.892, P = 0.003), proportion of patients with WBC count ≥ 50 × 10⁶/L (χ² = 22.503, P = 0.000), proportion of eosinophils (χ² = 13.551, P = 0.001), protein quatification (χ² = 20.391, P = 0.000), glucose level (χ² = 8.653, P = 0.013), proportion of patients with glucose < 2.20 mmol/L (Fisher′s exact probability: P = 0.010), and IgG (χ² = 13.899, P = 0.001) were statistically significant. Further pairwise comparisons revealed that, compared with the NMOSDs group and the MOGAD group, the GFAP-A group had a higher CSF pressure (t = -80.012, P = 0.000; t = -61.222, P = 0.002), a higher WBC count (Z = -3.178, P = 0.001; Z = -2.618, P = 0.009), a higher proportion of cases with WBC count ≥ 50 × 10⁶/L (Z = -4.163, P = 0.000; Z = -3.173, P = 0.002), higher level of protein quantification (Z = -4.193, P = 0.000; Z = -3.373, P = 0.001) and IgG (Z = -2.925, P = 0.003; Z = -3.375, P = 0.001), and a lower glucose level (Z = 2.433, P = 0.015; Z = 2.561, P = 0.010). Compared with the NMOSDs group, the GFAP-A group had a higher proportion of cases with eosinophils (Z = 3.643, P = 0.000) and a lower proportion of glucose < 2.20 mmol/L (Z = 2.795, P = 0.005). Conclusions: The CSF of NMOSDs and MOGAD patients is similar in terms of pressure, cytology and biochemistry. Compared with NMOSDs and MOGAD, the CSF pressure and WBC count of GFAP-A patients are higher, eosinophil infiltration is more common, protein quatification and IgG levels are significantly increased, and glucose levels are generally reduced. These features may aid in the diagnosis and differential diagnosis of GFAP-A.

Key words: Demyelinating autoimmune diseases, CNS, Cerebrospinal fluid, Leukocyte count, Proteins

摘要：

目的: 对比抗水通道蛋白4（AQP4）抗体阳性的视神经脊髓炎谱系疾病（NMOSDs）、髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）和自身免疫性胶质纤维酸性蛋白星形细胞病（GFAP-A）的脑脊液特征，为其诊断与鉴别诊断提供参考。方法与结果: 选择2019年4月至2023年8月宁夏医科大学总医院收治的血清抗AQP4抗体阳性NMOSDs患者38例、MOGAD患者14例、GFAP-A患者27例。收集3组患者临床资料以及脑脊液压力、细胞学、生化、IgG等。NMOSDs组、MOGAD组与GFAP-A组相比，脑脊液压力（F=10.530，P=0.000）、白细胞计数（χ²=11.892，P=0.003）、白细胞计数≥ 50 × 10⁶/L者占比（χ²=22.503，P=0.000）、存在嗜酸性粒细胞者占比（χ²=13.551，P=0.001）、蛋白定量（χ²=20.391，P=0.000）、葡萄糖（χ²=8.635，P=0.013）、葡萄糖 < 2.20 mmol/L者占比（Fisher确切概率法：P=0.010）以及IgG（χ²=13.899，P=0.001）差异均有统计学意义。进一步两两比较，与NMOSDs组和MOGAD组相比，GFAP-A组脑脊液压力较高（t=- 80.012，P=0.000；t=- 61.222，P=0.002），白细胞计数（Z =- 3.178，P=0.001；Z =- 2.618，P=0.009）和白细胞计数≥ 50 × 10⁶/L者占比较高（Z =- 4.163，P=0.000；Z =- 3.173，P=0.002），蛋白定量（Z =- 4.193，P=0.000；Z =- 3.373，P=0.001）和IgG（Z =- 2.925，P=0.003；Z =- 3.375，P=0.001）较高，葡萄糖水平较低（Z =2.433，P=0.015；Z =2.561，P=0.010）；与NMOSDs组相比，GFAP-A组存在嗜酸性粒细胞者占比较高（Z =3.643，P=0.000），葡萄糖 < 2.20 mmol/L者占比较低（Z =2.795，P=0.005）。结论: NMOSDs与MOGAD患者在脑脊液压力、细胞学、生化方面表现相似，相较于NMOSDs和MOGAD，GFAP-A患者脑脊液压力和白细胞计数更高，嗜酸性粒细胞浸润多见，蛋白定量和IgG升高，葡萄糖水平降低，可辅助诊断与鉴别诊断。

关键词: 脱髓鞘自身免疫疾病，中枢神经, 脑脊髓液, 白细胞计数, 蛋白质类

Yue YANG, Gao-jie XUE, Lu LIU, Yan-bai WANG, Yu-ting HOU, Xiao YANG. Comparison of cerebrospinal fluid characteristics in three rare types of autoimmune inflammatory demyelinating diseases[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2025, 25(12): 1121-1127.

杨悦, 薛高杰, 刘露, 王妍柏, 侯玉婷, 杨笑. 三种罕见类型自身免疫性炎性脱髓鞘疾病患者脑脊液特征对比分析[J]. 中国现代神经疾病杂志, 2025, 25(12): 1121-1127.

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观察指标	NMOSDs组（n = 38）	MOGAD组（n = 14）	GFAP-A组（n = 27）	统计量值	P值
脑脊液
压力（${\bar x}$ ± s，mm H₂O）	123.21±44.89	142.00±35.80	203.22±81.82	10.530	0.000
白细胞计数[M（P₂₅，P₇₅），×10⁶/L]	15.00（6.50，22.75）	18.50（2.00，25.00）	87.00（10.00，225.00）	11.892	0.003
白细胞计数≥ 50×10⁶/L[例（%）]	4（10.53）	1（1/14）	16（59.26）	22.503	0.000
白细胞计数升高[例（%）]	27（71.05）	8（8/14）	21（77.78）	1.903	0.386
存在嗜酸性粒细胞[例（%）]	4（14.81）	3（3/8）	14（66.67）	13.551	0.001
中性粒细胞比例[M（P₂₅，P₇₅），%]	1.00（0.00，12.00）	12.00（2.00，14.00）	0.00（0.00，2.50）	2.582	0.275
蛋白定量[M（P₂₅，P₇₅），mg/L]	530.00（360.00，920.00）	520.00（370.00，790.00）	1500.00（700.00，2140.00）	20.391	0.000
葡萄糖[M（P₂₅，P₇₅），mmol/L]	3.00（2.78，3.20）	3.20（2.58，3.63）	2.40（2.10，3.00）	8.635	0.013
葡萄糖 < 2.20 mmol/L[例（%）]	1（2.63）	1（1/14）	7（25.93）	—	0.010
IgG[M（P₂₅，P₇₅），mg/L]	39.95（31.85，90.75）	40.15（18.45，49.83）	80.00（54.30，150.00）	13.899	0.001
血糖[M（P₂₅，P₇₅），mmol/L]	6.20（5.20，6.80）	6.00（5.30，7.00）	6.70（5.90，7.50）	4.470	0.107

观察指标	NMOSDs组（n = 38）	MOGAD组（n = 14）	GFAP-A组（n = 27）	统计量值	P值
脑脊液
压力（${\bar x}$ ± s，mm H₂O）	123.21±44.89	142.00±35.80	203.22±81.82	10.530	0.000
白细胞计数[M（P₂₅，P₇₅），×10⁶/L]	15.00（6.50，22.75）	18.50（2.00，25.00）	87.00（10.00，225.00）	11.892	0.003
白细胞计数≥ 50×10⁶/L[例（%）]	4（10.53）	1（1/14）	16（59.26）	22.503	0.000
白细胞计数升高[例（%）]	27（71.05）	8（8/14）	21（77.78）	1.903	0.386
存在嗜酸性粒细胞[例（%）]	4（14.81）	3（3/8）	14（66.67）	13.551	0.001
中性粒细胞比例[M（P₂₅，P₇₅），%]	1.00（0.00，12.00）	12.00（2.00，14.00）	0.00（0.00，2.50）	2.582	0.275
蛋白定量[M（P₂₅，P₇₅），mg/L]	530.00（360.00，920.00）	520.00（370.00，790.00）	1500.00（700.00，2140.00）	20.391	0.000
葡萄糖[M（P₂₅，P₇₅），mmol/L]	3.00（2.78，3.20）	3.20（2.58，3.63）	2.40（2.10，3.00）	8.635	0.013
葡萄糖 < 2.20 mmol/L[例（%）]	1（2.63）	1（1/14）	7（25.93）	—	0.010
IgG[M（P₂₅，P₇₅），mg/L]	39.95（31.85，90.75）	40.15（18.45，49.83）	80.00（54.30，150.00）	13.899	0.001
血糖[M（P₂₅，P₇₅），mmol/L]	6.20（5.20，6.80）	6.00（5.30，7.00）	6.70（5.90，7.50）	4.470	0.107

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