Comparison of clinical and radiological characteristics in autoimmune GFAP astrocytopathy, MOGAD and AQP4-IgG+ NMOSD mimicking intracranial infection as the initial manifestation
Comparison of clinical and radiological characteristics in autoimmune GFAP astrocytopathy, MOGAD and AQP4-IgG+ NMOSD mimicking intracranial infection as the initial manifestation
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以颅内感染为首发表现的自身免疫性 GFAP 星形细胞病、MOGAD 和 AQP4-IgG NMOSD 的临床和影像学特征比较
DOI：
10.1016/j.msard.2022.104057
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发表时间：
2022
影响因子：
4
通讯作者：
Dai-Shi Tian
中科院分区：
文献类型：
--
作者：
Jun Xiao;Shuo-Qi Zhang;Xin Chen;Yue Tang;Man Chen;Ke Shang;Gang Deng;Chuan Qin;Dai-Shi Tian
摘要目的研究自身免疫性神经胶质酸性蛋白星形细胞病等多种自身免疫性中枢神经系统炎症性疾病（A-GFAP-A）、髓磷脂少突胶质细胞糖蛋白抗体相关疾病（MOGAD）和水通道蛋白-4-免疫球蛋白-G-阳性视神经肌萎缩谱系疾病（AQP 4-IgG+NMOSD）最初通常表现为类似颅内感染的症状，在缺乏自身抗体检测的早发期不易鉴别。方法回顾性分析以颅内感染为首发症状的A-GFAP-A型9例，MOGAD型17例，AQP 4-IgG+NMOSD型11例。通过基于细胞的测定来检测自身抗体。总结了其临床、免疫学及影像学特点。结果在队列中，震颤和阳性Kernig征在A-GFAP-A中占主导地位（分别为44.4%和77.8%），超过MOGAD（5.9%，p = 0.034; 29.4%，p = 0.038）和AQP 4-IgG+NMOSD（0，p = 0.026; 18.2%，p = 0.022）。10例患者（A-GFAP-A 4例，MOGAD 5例，AQP 4-IgG+NMOSD 1例）最初被误诊为结核性或病毒性脑膜脑炎，但对经验性抗结核或抗病毒治疗耐药，经调整治疗后免疫治疗部分或完全缓解。在脑脊液（CSF）检查中，A-GFAP-A队列（中位数，90×106/L [IQR，41-209]）中CSF中的白色血细胞计数高于AQP 4-IgG+ NMOSD（中位数，6 × 106/L [IQR，p = 0.018）。重要的是，CSF蛋白的增加越高，（1319 mg/L [IQR，1035-1519），乳酸脱氢酶（LDH，53.9 ± 37.2 U/L），乳酸（3.50 ± 0.88 mmol/L），IgG（130.9 ± 60.4 mg/L），IgM（8.6 ± 6.1 mg/L）和伊加A-GFAP-A组（23.0 ± 11.4 mg/L）高于MOGAD组（P <0.01），差异有统计学意义（P <0.05）。（CSF蛋白：441 mg/L [IQR，p = 0.004; LDH：53.9 ± 37.2 U/L，p = 0.005;乳酸：2.15 ± 0.62 mmol/L，p = 0.001; IgG：77.9 ± 71.3 mg/L，p = 0.018; IgM，2.7 ± 2.9 mg/L，p = 0.015）和AQP4-IgG+ NMOSD（CSF蛋白：386 mg/L [IQR，p = 0.002; LDH：23.7 ± 11.0 U/L，p = 0.048;乳酸：2.40 ± 0.66 mmol/L，p = 0.040; IgG：53.2 ± 30.3 mg/L，p = 0.015; IgM，2.1 ± 3.9 mg/L，p = 0.004;伊加，5.2 ± 5.0 mg/L，p = 0.001）和AQP4-IgG+NMOSD（0%，p = 0.026）。此外，与MOGAD（5，29.4%，p = 0.011）和AQP 4-IgG+NMOSD（1/6，16.7%，p = 0.011）相比，A-GFAP-A（8，88.9%）中弥漫性脑膜增强更常见。
AbstractObjective Several autoimmune CNS inflammatory diseases, including autoimmune glial fibrillary acidic protein astrocytopathy (A-GFAP-A), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and aquaporin-4-immunoglobulin-G-positive neuromyelitis optica spectrum disorders (AQP4-IgG+NMOSD) often presented initially with similar symptoms mimicking intracranial infection, are not easy to be differentiated during early-onset lacking the detection of autoantibody. Methods In our single-center cohorts, those patients mimicking intracranial infection as initial symptoms, including 9 with A-GFAP-A, 17 with MOGAD and 11 with AQP4-IgG+NMOSD, were retrospectively included. The autoantibodies were detected by cell-based assays. The clinical, immunological and radiological characteristics were summarized. Results In the cohort, tremor and positive Kernig's sign were predominated in A-GFAP-A (44.4% and 77.8%, respectively) over MOGAD (5.9%, p = 0.034; 29.4%, p = 0.038) and AQP4-IgG+NMOSD (0, p = 0.026; 18.2%, p = 0.022). Ten patients (A-GFAP-A, 4; MOGAD, 5; AQP4-IgG+NMOSD, 1) were initially misdiagnosed as tubercular or viral meningoencephalitis, however, resistant to empiric anti-tuberculosis or anti-viral treatment, and finally were in partial or complete remission with the immunotherapy when adjusted treatments. On cerebrospinal fluid (CSF) examination, white blood cell counts in CSF was higher in A-GFAP-A cohort (median, 90×106/L [IQR, 41-209) compared to AQP4-IgG+ NMOSD (median, 6 × 106/L [IQR, p = 0.018). Importantly, the higher increase in CSF protein (1319 mg/L [IQR, 1035-1519), lactate dehydrogenase (LDH, 53.9 ± 37.2 U/L), lactic acid (3.50 ± 0.88 mmol/L), IgG (130.9 ± 60.4 mg/L), IgM (8.6 ± 6.1 mg/L) and IgA (23.0 ± 11.4 mg/L) levels in A-GFAP-A was found compared to MOGAD (CSF protein: 441 mg/L [IQR, p = 0.004; LDH: 53.9 ± 37.2 U/L, p = 0.005; lactic acid: 2.15 ± 0.62 mmol/L, p = 0.001; IgG: 77.9 ± 71.3 mg/L, p = 0.018; IgM, 2.7 ± 2.9 mg/L, p = 0.015) and AQP4-IgG+ NMOSD (CSF protein: 386 mg/L [IQR, p = 0.002; LDH: 23.7 ± 11.0 U/L, p = 0.048; lactic acid: 2.40 ± 0.66 mmol/L, p = 0.040; IgG, 53.2 ± 30.3 mg/L, p = 0.015; IgM, 2.1 ± 3.9 mg/L, p = 0.004; IgA, 5.2 ± 5.0 mg/L, p p = 0.001) and AQP4-IgG+NMOSD (0%, p = 0.026). In addition, diffuse meningeal enhancement was more common in A-GFAP-A (8, 88.9%) compared to MOGAD (5, 29.4%, p = 0.011) and AQP4-IgG+NMOSD (1/6, 16.7%, p = 0.011), respectively.