目的 探讨自身免疫性胶质纤维酸性蛋白星形细胞病的脑膜脊膜病变特点,旨在提高临床医师对自身免疫性胶质纤维酸性蛋白星形细胞病(GFAP-A)的认识与诊治.方法 一项回顾性病例对照研究.通过免疫荧光的方法检测出阳性的GFAP-IgG 55例,其中28例患者完成头颅MRI的平扫和增强(25例同时完成脊髓MRI平扫和增强).对照组为同期住院的27例AQP4-IgG阳性的视神经脊髓炎谱系疾病患者,均完成头颅和脊髓MRI平扫和增强.结果 纳入28例GFAP-A,主要临床症状包括头痛(46.4％,13/28)、发热(57.1％,16/28)、脊髓炎(42.9％,12/28)、视觉异常(39.3％,11/28)、行为异常(28.6％,8/28)、共济失调(25.0％,7/28)、意识障碍(14.3％,4/28)、癫痫发作(10.7％,3/28)、运动障碍(7.1％,2/28)、认知障碍(10.7％,3/28)和其他症状(25％,7/28).MRI增强下15例(53.6％)出现脑膜和(或)脊膜的异常信号,其中脑膜异常8例(28.6％),脊膜异常3例(10.7％),脑膜与脊膜同时异常4例(14.3％).两例病理资料显示脑膜增厚,慢性炎症改变.与视神经脊髓炎谱系疾病对比显示性别比例、发热、头痛、行为异常、视觉异常、MRI放射性血管样强化、脊髓异常、长节段脊髓炎、脑膜和(或)脊膜异常差异均有统计学意义(P<0.05).

目的 探讨自身免疫性GFAP星型细胞病的脑电图(electroencephalographic,EEG)的特点.方法 对确诊自身免疫性GFAP星型细胞病患者的临床和脑电图等资料进行回顾性分析.分析不同临床表现患者脑电图、头MR影像特点,随访3～12个月.结果 共纳入自身免疫性GFAP星型细胞病患者38例,主要临床表现包括肢体乏力、麻木(n=20,52.6％)、发热(n=14,42.1％)、头痛(n=16,42.1％)、排便障碍(n=9,23.7％)、癫痫发作(n=5,13.2％).26例患者有效脑电图记录中3例可见各导联弥漫性慢活动出现,5例出现癫痫样放电,9例出现散在慢波,9例正常脑电图.结论 EEG对自身免疫性GFAP星型细胞病的诊断、追踪随访及预后评估有实用意义.

Objective To analyze the magnetic resonance imaging (MRI) of the spinal cord and clinical characteristics in patients with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy.Methods A total of 1 040 samples of cerebrospinal fluid (CSF) and sera collected in the Second Affiliated Hospital of Guangzhou Medical University from March 2013 to June 2018 were tested with tissue-and cell-based assays,and 42 patients were found positive for GFAP-IgG.The clinical data and MRI characteristics of the spinal cord of 19 patients who were positive for GFAP-IgG in CSF with autoimmune GFAP astrocytopathy and lesions in the spinal cord were retrospectively reviewed.Results There were 12 females and seven males among the 19 patients,with onset age of (44±17) years.The main manifestations of these patients included limb weakness (14/19),abnormal vision (5/19),headache (4/19),seizure (4/19),dementia (3/19),etc.On MRI of the spinal cord,five patients showed involvement in the cervical cord alone,eight showed involvement in the thoracic cord alone and six had both cervical and thoracic segment involvement.Fifteen patients had longitudinally extensive myelitic abnormalities (≥3 vertebral segments long).Seven enhancement patterns were encountered.Lesions were displayed in the spinal cord and brain in eight patients.Central gray matter involvement in the spinal cord was found in all the 19 patients.Conclusions Autoimmune GFAP astrocytopathy more frequently presents in females than in males.MRI of the spinal cord has complex presentations and longitudinally extensive myelitic abnormalities usually.Patients often show central gray matter involvement in the spinal cord.Myelitic abnormalities present more often in thoracic segment than in cervical segment.Abnormalities in lumbar segment are less encountered.

BACKGROUND:In this study, we describe clinical findings in a patient with autoimmune inflammatory meningoencephalitis who was negative for antibodies against glial fibrillary acidic protein (GFAP-IgG).METHODS:Serum and cerebral spinal fluid (CSF) samples were collected from the patient as part of a study of 520 patients with neurological syndromes. Antibodies against GFAP and other proteins associated with neurological disorders were measured by rat brain- and cell-based indirect immunofluorescence assays.RESULTS:A 42-year-old female was diagnosed with autoimmune inflammatory meningoencephalitis. She experienced a subacute and relapsing course with decreased vision, fever, headache, ataxia, hemiplegia, and disturbance of consciousness. Brain magnetic resonance imaging showed extensive lesions in the white matter along the ventricle, brainstem, right internal capsule, and meninges. The patient responded well to steroid treatment. Examination of CSF revealed a normal white blood cell count and protein level. Serum and CSF were negative for GFAP-specific antibodies and all other autoantibodies tested. Immunohistochemical staining of a brain biopsy collected during relapse revealed chronic inflammation and severe edema. Extensive and strong staining of CD163+ macrophages were evident throughout the lesions; however, CD3+ cells were rare and CD138+ and CD20+ cells were absent.CONCLUSION:We describe a case of subacute corticosteroid-responsive nonvasculitic autoimmune inflammatory meningoencephalitis in the absence of GFAP-IgG. The pathological features were distinct from those of patients with GFAP-IgG-positive meningoencephalitis, suggesting that nonvasculitic autoimmune inflammatory meningoencephalitis is a heterogeneous neurological syndrome.

Aim: To investigate the clinical features of five glial fibrillary acidic protein (GFAP) antibody positive patients with suspected benign tumors and explore its underlying pathogenesis. Materials and methods: Overall, 1018 serum and cerebrospinal fluid (CSF) samples were tested by indirect immunofluorescence assay and data from five patients with suspected tumors and positive for GFAP autoantibody in the CSF were analyzed retrospectively. Results: The positive rate of GFAP antibody in the serum and CSF was 3.93% by indirect immunofluorescence assay. Tumors were diagnosed before or after neurologic onset in 5 of 40 patients (12.5%) and no deterioration of the tumors was found during the long-term follow-up. Of the five patients, one patient suffered a thyroid nodule, one patient had a small nodule in the left lung, two patients suffered meningiomas, and one patient had a suspicious eosinophilic granuloma. Conclusion: GFAP autoimmunity may be a paraneoplastic immune response with a low frequency of tumor in Chinese patients with GFAP astrocytopathy.

Objective: Determination of glial fibrillary acidic protein (GFAP), aquaporin 4 (AQP4), and myelin oligodendrocyte glycoprotein (MOG) levels in cerebrospinal fluid (CSF), and astrocytic damage analysis in patients with GFAP astrocytopathy (GFAP-A) and other conditions. Methods: GFAP, AQP4, and MOG levels in CSF were detected via enzyme-linked immunosorbent assays. Anti-GFAP, anti-AQP4, and anti-MOG IgGs were detected via indirect immunofluorescence assays. Results: In 32 GFAP-Astrocytopathy patients, CSF GFAP was significantly higher during acute exacerbation than it was in patients with MOG encephalomyelitis, multiple sclerosis, autoimmune encephalitis, and an "other inflammatory neurological disorders" group (all p < 0.0001). CSF GFAP levels were slightly higher in the GFAP-A group than in an anti-AQP4 IgG-positive neuromyelitis optica spectrum disorder group (p = 0.012). There were no significant differences between the CSF MOG and AQP4 levels in the GFAP-A group and those of other groups. CSF GFAP levels were significantly reduced after steroid treatment (p = 0.011). CSF GFAP levels differed significantly in GFAP-Astrocytopathy patients with and without encephalitis (p = 0.016). In GFAP-Astrocytopathy patients, CSF GFAP was correlated with Expanded Disability Status Scale (EDSS) score during attack (r = 0.545, p = 0.001). In follow-up examinations however, in GFAP-Astrocytopathy patients CSF GFAP level was not correlated with EDSS score 6 months later. Conclusions: CSF GFAP level and pathological examination of GFAP-Astrocytopathy patients revealed astrocyte damage. CSF GFAP level was associated with steroid treatment at the acute stage, therefore CSF GFAP may be a sensitive biomarker with respect to the effects of therapy during the acute stage.

Objective: The aim of this study was to evaluate the positive rate of serum glutamic acid decarboxylase (GAD) autoantibody in patients with myelitis and to describe the clinical findings in patients with positive GAD antibody. Methods: Serum samples were collected from 390 patients with myelitis, including 210 patients positive for aquaporin 4 (AQP4) antibody and 180 patients negative for AQP4. GAD65 antibody was measured by an indirect immunofluorescence assay. Results: Only 1 serum and cerebral spinal fluid sample from 390 patients (0.26%) was positive for anti-GAD antibodies. The patient was a female with relapsing myelitis and a thymic mass. Thymic resection was undertaken, and pathological examination revealed a benign thymic cyst. Extensive infiltration of lymphocytes positive for CD3, CD4, CD8 and CD20 was found. Immunohistochemistry showed positive expression of GAD65 in the cyst. Conclusions: Although serum GAD65 antibodies were present in a patient, it is not recommended to routinely screen for GAD65 antibodies in patients with myelitis because of their rare occurrence. However, screening for GAD65 antibodies should be considered in patients who have been diagnosed with cancer or a thymic abnormality.

BACKGROUND:To evaluate indirect immunofluorescence patterns of auto-antibodies and the targeting antigens to Immunoglobulin Gs (IgGs) in the cerebrospinal fluid (CSF) by a tissue-based assay(TBA).METHODS:CSF samples were collected from 793 patients. Auto-antibody levels were measured via an immunofluorescence assay.RESULTS:110 (13.9%) CSF samples with a specific response were confirmed. Of these, 37 showed a neuronal pattern, 57 an astrocyte pattern, 7 a neuronal and astrocyte pattern, and 9 samples showed an oligodendrocyte pattern. In the neuronal antibody group, 16 patients had NMDAR-IgGs, 3 had LGi1-IgGs, 2 had AMPA2-IgGs, 2 had GAD65-IgGs, 1 patient had GABA-IgGs, and 1 patient had overlapping NMDAR-IgGs and AQP4-IgGs. Of the unidentified neuronal antibodies, two were cellular surface antibodies, three were cellular surface and cytoplasm antibodies, three were cytoplasm antibodies, and four were nuclear and cytoplasm antibodies. Among the 57 patients with the astrocyte pattern, 28 patients were positive for AQP4-IgGs, 21 were positive for GFAP-IgGs, 5 patients had overlapping AQP4 and GFAP-IgGs, and 3 patients had an unidentified antigen. Seven patients showed neuronal and astrocyte patterns simultaneously; four of them had unknown neuronal antibodies. In the patients with an oligodendrocyte pattern, one was positive for MOG-IgGs and four for MBP-IgGs.CONCLUSIONS:The TBA is helpful for diagnosing autoimmune neurological syndrome, especially in patients with unknown antibodies and antigens. Presence of unidentified antibodies against neuronal or glial cells could be an interesting finding, but should be investigated in future studies which incorporate parallel serum samples at an appropriate IgG dilution.

Objective Our study aimed to delineate the clinical and radiological features of patients with anti-myelin oligodendrocyte glycoprotein antibody (MOG-Ab)positive neuromyelitis optica spectrum disorder (NMOSD). Methods Fifty-seven patients with NMOSD and 29 patients with multiple sclerosis (MS) were collected. Data on clinical and radiological features of MOG-Ab positive patients with were analyzed retrospectively. Results MOG-Abs were present in 9/57 (15.8%) NMOSD patients and 2/29 (6.9%) MS patients. Both MOG and aquaporin-4 (AQP4) antibodies were positive in one case of NMOSD. There was no significant difference between the two groups (P＞0.05). There were more females than males having MOG-Ab positive NMOSD (females: males=7:1) and the average onset age was 41.4 ± 11.5 years. There was no significant difference in gender and age between MOG-Ab negative and AQP4-Ab positive groups(P＞0.05). The durations of disease were significantly shorter in either MOG-Ab positive NMOSD patients or MOG-Ab negative NMOSD patients than in AQP4-Ab positive group (P<0.05). Recurrence was the main disease pattern of all three groups and the frequency of recurrence was not significant different among three groups (P＞0.05). The incidence of optic neuritis was 62.5% in NMOSD patients with MOG-Ab positive and 43.5% in AQP4-Ab positive NMOSD patients (P＞0.05). There was no significant difference in the morphology and location of brain lesions among the three groups (P＞0.05). MOG-Ab positive NMOSD patients had long segment spinal cord lesions. The median length of the spinal cord lesions in the MOG-Ab positive group was similar to the other two groups (P＞0.05). Conclusions MOG-Ab positive NMOSD patients have higher proportion of females with shorter recurrence course, more likely complicated with optic neuritis. And the radiological features of brain and spinal cord were not specific to patients with AQP4-Ab positive.