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1.
Curr Neurovasc Res ; 2022 May 13.
Artigo em Inglês | MEDLINE | ID: mdl-35570518

RESUMO

OBJECTIVES: Coronary artery stenosis (CAS) ≥50% often coexists in patients with ischemic stroke, which leads to a significant increase in the occurrence of major vascular events after stroke. This study aimed to develop a nomogram for diagnosing presence of ≥50% asymptomatic CAS in patients with ischemic stroke. METHODS: A primary cohort was established that included 275 non-cardioembolic ischemic stroke patients who were admitted from January 2011 to April 2013 in a teaching hospital in southern China. The preoperative data were used to construct two models by the best subset regression and the forward stepwise regression methods, and a nomogram between these models was established. The assessment of the nomogram was carried out by discrimination and calibration in an internal cohort. RESULTS: Out of the two models, model 1 contained eight clinical-related variables, and exhibited the lowest Akaike Information Criterion value (322.26) and highest concordance index 0.716 (95% CI, 0.654-0.778). The nomogram showed good calibration and significant clinical benefit according to calibration curves and the decision curve analysis. CONCLUSION: The nomogram, composed of age, sex, NIHSS score on admission, hypertension history, fast glucose level, HDL cholesterol level, LDL cholesterol level, and presence of ≥50% cervicocephalic artery stenosis, can be used for prediction of ≥50% asymptomatic coronary artery disease (CAD). Further studies are needed to validate the effectiveness of this nomogram in other populations.

2.
Mult Scler Relat Disord ; 59: 103527, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35172264

RESUMO

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is a rare and severe inflammatory demyelinating disorder of the central nervous system (CNS), which mainly affects the optic nerves and spinal cord. The aims of this study were to determine whether the expression levels of serological cytokines could distinguish 1) NMOSD from healthy controls (HCs); and 2) NMOSD patients with and without the aquaporin-4 (AQP4) antibody biomarker from each other; and 3) NMOSD patients without the antibody to AQP4 from MS patients. METHODS: The expression levels of 200 proteins in serum from 41 NMOSD (32 with antibodies to AQP4, 9 without antibodies to AQP4), 12 MS patients, and 34 HCs were measured using glass-based antibody arrays. None of the patients received any immunosuppressive treatment. In parallel, the correlation between protein expression in NMOSD/MS patients and clinical traits was determined with Weighted Gene Co-expression Network Analysis (WGCNA). RESULTS: Thirty-nine serological proteins were differentially expressed in NMOSD patients compared to HCs, with 29 of these proteins not observed in MS patients. In addition, the data reveal 15 differentially-expression proteins (DEPs) between AQP4-IgG seronegative and AQP4-IgG seropositive NMOSD patients, and 9 DEPs between NMOSD and MS patients who did not have AQP4-IgG. CONCLUSION: Serological IL-17B is significantly upregulated in both NMOSD and MS patients compared to HCs, and could be a key biomarker of NMOSD and MS. Serological VEGF, MPIF-1 and NrCAM were positively associated with AQP4-IgG titer. We also demonstrate that EGF may be involved in the breakdown of the BBB by downregulating Claudin-5.


Assuntos
Esclerose Múltipla , Neuromielite Óptica , Aquaporina 4 , Autoanticorpos , Biomarcadores , Humanos , Esclerose Múltipla/complicações , Esclerose Múltipla/diagnóstico , Neuromielite Óptica/complicações
3.
J Neurointerv Surg ; 2021 Dec 02.
Artigo em Inglês | MEDLINE | ID: mdl-34857667

RESUMO

BACKGROUND: Mechanical thrombectomy is the standard treatment for acute ischemic stroke (AIS) with large vessel occlusion (LVO) in the anterior circulation. This trial aimed to indicate whether Skyflow, a new thrombectomy device, could achieve the same safety and efficacy as Solitaire FR in the treatment of AIS. METHODS: This study was a prospective, multicenter, randomized, single blind, parallel, positive controlled, non-inferiority clinical trial. Patients with intracranial anterior circulation LVO within 8 hours from onset were included to receive thrombectomy treatment with either the Skyflow or Solitaire FR stent retriever. The primary endpoint was the rate of successful reperfusion (modified Treatment In Cerebral Infarction (mTICI) ≥2b) after the operation. The safety endpoints were the rate of symptomatic intracranial hemorrhage (sICH) and subarachnoid hemorrhage (SAH) at 24 hours after operation. RESULTS: A total of 95 and 97 patients were involved in the Skyflow group and Solitaire FR group, respectively. A successful reperfusion (mTICI ≥2b) was finally achieved in 84 (88.4%) patients in the Skyflow group and 80 (82.5%) patients in the Solitaire FR group. Skyflow was non-inferior to Solitaire FR in regard to the primary outcome, with the criterion of a non-inferiority margin of 12.5% (p=0.0002) after being adjusted for the combined center effect and the National Institutes of Health Stroke Scale (NIHSS) score. The rate of periprocedural sICH and SAH did not differ significantly between the two groups. CONCLUSION: Endovascular thrombectomy with the Skyflow stent retriever was non-inferior to Solitaire FR with regard to successful reperfusion in AIS due to LVO (with a pre-specified non-inferiority margin of 12.5%).

4.
Front Immunol ; 12: 720907, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34421925

RESUMO

Objective: To explore the outcomes of NMOSD attacks and investigate serum biomarkers for prognosis and severity. Method: Patients with NMOSD attacks were prospectively and observationally enrolled from January 2019 to December 2020 at four hospitals in Guangzhou, southern China. Data were collected at attack, discharge and 1/3/6 months after acute treatment. Serum cytokine/chemokine and neurofilament light chain (NfL) levels were examined at the onset stage. Results: One hundred patients with NMOSD attacks were included. The treatment comprised intravenous methylprednisolone pulse therapy alone (IVMP, 71%), IVMP combined with apheresis (8%), IVMP combined with intravenous immunoglobulin (18%) and other therapies (3%). EDSS scores decreased significantly from a medium of 4 (interquartile range 3.0-5.5) at attack to 3.5 (3.0-4.5) at discharge, 3.5 (2.0-4.0) at the 1-month visit and 3.0 (2.0-4.0) at the 3-month visit (p<0.01 in all comparisons). The remission rate was 38.0% at discharge and 63.3% at the 1-month visit. Notably, relapse occurred in 12.2% of 74 patients by the 6-month follow-up. Higher levels of T helper cell 2 (Th2)-related cytokines, including interleukin (IL)-4, IL-10, IL-13, and IL-1 receptor antagonist, predicted remission at the 1-month visit (OR=9.33, p=0.04). Serum NfL levels correlated positively with onset EDSS scores in acute-phase NMOSD (p<0.001, R2 = 0.487). Conclusions: Outcomes of NMOSD attacks were generally moderate. A high level of serum Th2-related cytokines predicted remission at the 1-month visit, and serum NfL may serve as a biomarker of disease severity at attack. Clinical Trial Registration: https://clinicaltrials.gov/ct2/show/NCT04101058, identifier NCT04101058.


Assuntos
Biomarcadores , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/terapia , Adulto , Biomarcadores/sangue , Citocinas/sangue , Gerenciamento Clínico , Progressão da Doença , Suscetibilidade a Doenças , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Proteínas de Neurofilamentos/sangue , Neuromielite Óptica/sangue , Neuromielite Óptica/etiologia , Gravidade do Paciente , Prognóstico , Estudos Prospectivos , Recidiva , Avaliação de Sintomas , Subpopulações de Linfócitos T/imunologia , Subpopulações de Linfócitos T/metabolismo , Resultado do Tratamento
5.
Front Neurol ; 12: 651511, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33897605

RESUMO

Objective: The prevalence of multiple sclerosis (MS) in China is low, although it has been increasing recently. Owing to the paucity of data on immunotherapy acceptance in the Chinese population, we conducted this study to analyze factors affecting the acceptance of immunotherapy and selection of disease-modifying therapies (DMTs) based on personal and clinical data of patients with MS. Methods: In this study, data were obtained from the Multiple Sclerosis Patient Survival Report 2018, which was the first national survey of patients with MS in China. There were 1,212 patients with MS from 31 provinces who were treated at 49 Chinese hospitals over a 4-month period from May 2018 to August 2018, and the patients were asked to complete online questionnaires to assess their understanding of the disease. Results: In general, highly educated patients with frequent relapses were more willing to receive treatment regardless of DMTs or other immunotherapy, and patients with more understanding of the disease opted to be treated. Younger patient population, patients with severe disease course, and those with more symptoms were likely to choose the treatment. Moreover, a higher proportion of women chose to be treated with DMTs than with other immunotherapies. Conclusions: Education status and patient awareness of the disease impact the treatment acceptance in Chinese patients with MS. Therefore, we call for improving the awareness of MS disease and social security to help patients to improve their quality of life.

6.
Eur J Neurol ; 28(6): 2121-2125, 2021 06.
Artigo em Inglês | MEDLINE | ID: mdl-33590610

RESUMO

BACKGROUND AND OBJECTIVE: We aimed to report the pathological features of T lymphocytes in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A). METHODS: A retrospective pathological analysis of patients with GFAP-A was performed. RESULTS: Eight patients with GFAP-immunoglobulin G (IgG) and pathological data were included. Their biopsy findings were similar, and all showed marked lymphocytic infiltration in the white matter, with perivascular predominance. The lymphocytic infiltration was predominantly composed of CD8+ T lymphocytes rather than CD4+ T lymphocytes, except in one patient who had overlapping positive myelin oligodendrocyte glycoprotein-IgG. Unlike CD4+ T cells, CD8+ T cells were frequently observed adjacent to dystrophic neurons and astrocytes. There was also diffuse infiltration by CD68+ and CD163+ macrophages. CD8+ astrocytes were identified in two samples, but no CD4+ astrocytes were observed. CONCLUSIONS: A predominance of CD8+ T cells may be an important pathological and diagnostic feature in GFAP-A.


Assuntos
Autoanticorpos , Linfócitos T CD8-Positivos , Proteína Glial Fibrilar Ácida , Humanos , Glicoproteína Mielina-Oligodendrócito , Estudos Retrospectivos
7.
Int J Neurosci ; : 1-6, 2020 Dec 30.
Artigo em Inglês | MEDLINE | ID: mdl-33287611

RESUMO

PURPOSE: This case report is the first to describe the detection of antibodies against inositol 1,4,5-trisphosphate receptor 1 (ITPR1, I3PR) in a patient diagnosed with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. ITPR1 is known as one of the Purkinje cell antibodies present in autoimmune cerebellar ataxia (ACA). Here, we described the association between autoimmune GFAP astrocytopathy and autoimmune cerebellar disease (ACD). MATERIALS AND METHODS: Demographic features, clinical characteristics, cerebrospinal fluid (CSF) parameters and neuroimaging findings were collected from this patient. Specifically, antibodies against GFAP and other proteins associated with neurological disorders were measured by immunofluorescence staining in both serum and CSF samples. RESULTS: A 52-year-old woman was diagnosed with autoimmune inflammatory meningoencephalitis. She presented with cognitive dysfunction, psychiatric/behavioral abnormalities and serious insomnia with subacute onset. Brain magnetic resonance imaging (MRI) showed bilateral hyperintensity in the semioval centers on axial images and perivascular linear enhancement oriented radially to the ventricles on sagittal images. GFAP-IgG, oligoclonal bands (OBs), N-methyl-D-aspartate receptor (NMDAR)-IgG and ITPR1-IgG co-existed in her CSF. She responded well to immunoglobulin and steroid treatments. CONCLUSION: Here, we describe the case of a patient with autoimmune GFAP astrocytopathy whose CSF was positive for ITPR1-IgG; however, she did not show typical ataxia manifestations or cerebellar lesions on her MRI scan. This suggests that ITPR1-IgG is not pathogenic, and the positivity of this antibody in CSF is probably associated with the presence of autoimmune inflammation.

8.
Mult Scler Relat Disord ; 45: 102350, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32645637

RESUMO

BACKGROUND: Autoimmune glial fibrillary acidic protein astrocytopathy (A-GFAP-A) has been recently characterized as a novel autoimmune central nervous system (CNS) disorder with GFAP antibody as the biomarker. However, nonspecific symptoms of A-GFAP-A contribute to misdiagnosis. CASE PRESENTATION: The patients presented with initial symptoms of fever, headache, and nuchal rigidity. Case 1 exhibited mild signs of irritability, active tendon reflexes, and dysuria; case 2 had transient loss of consciousness. Cerebrospinal fluid (CSF) revealed lymphocytosis, elevated protein level, and decreased glucose level. Magnetic resonance imaging (MRI) revealed radial gadolinium enhancement perpendicular to the lateral ventricle. Viral meningitis or tubercular meningitis was suspected. However, their inflammatory and pathogenic indicators showed no abnormal changes, and empirical antibiotic and antiviral drugs did not result in remarkable recovery. Subsequently, cases were detected with a strongly positive expression of GFAP antibody in CSF and the symptoms improved dramatically after high-dose methylprednisolone pulse treatment. CONCLUSION: A-GFAP-A with meningitis-like symptoms could initially masquerade as intracranial infection, and prompt detection of GFAP antibody is essential for differentiation.


Assuntos
Meios de Contraste , Meningite , Astrócitos , Gadolínio , Proteína Glial Fibrilar Ácida , Humanos , Meningite/diagnóstico , Meningite/tratamento farmacológico
9.
Ther Adv Neurol Disord ; 13: 1756286420909973, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32547640

RESUMO

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy has been considered a novel central nervous system autoimmune disease characterized by relapse and responsiveness to corticosteroid with a specific GFAP-Immunoglobulin G (IgG) being noted in cerebrospinal fluid. We report the case of a 21-year-old girl presenting with dysuria and weariness, who subsequently developed blurry vision, slight dysphagia, slurred speech, and sensory abnormality. GFAP-IgG was detected in her cerebrospinal fluid. Magnetic resonance imaging using both T2-weighted and contrast-enhanced T1-weighted images revealed a rare finding of lesions distributed mainly in the entire spinal cord rather than typical brain lesions. After treating with corticosteroids, her clinical symptoms were alleviated, and the spinal cord lesion enhancement was reduced. Our observations extend the clinical spectrum of autoimmune GFAP astrocytopathy. We suggest that rare distributed lesions in the entire spinal cord in patients with autoimmune GFAP astrocytopathy cannot be ignored by neurologists. The identification of potential atypical lesions broadens the understanding of autoimmune GFAP astrocytopathy.

10.
Int J Neurosci ; 130(2): 186-192, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31696761

RESUMO

Background and Purpose: Oxidative stress is involved in the development of infections. However, whether oxidative stress indicators can be used as markers of stroke-associated infection (SAI) is still unclear. The purpose of this study was to test the predictive values of superoxide dismutase (SOD) and malondialdehyde (MDA) levels for SAI incidence.Methods: A total of 45 consecutive patients with ischemic stroke who were admitted to our hospital were enrolled. A prospective study was carried out to observe the occurrence of SAI during the first 7 days after stroke. Accordingly, the patients were divided into SAI and non-SAI groups. The relationship between SOD and MDA serum levels and SAI was analyzed.Results: The patients in the SAI group had significantly higher serum SOD levels than those in the non-SAI group (41.638 ± 3.428 U/ml vs. 36.542 ± 9.114 U/ml, p = 0.033). However, there were no significant differences in MDA levels between the SAI and non-SAI group (p > 0.05). The discriminating ability of serum SOD level for SAI was measured using an ROC curve. Serum level of SOD >38.16 U/ml was useful in diagnosing SAI with a sensitivity of 88% and a specificity of 61%. Kaplan-Meier curves showed that the group with serum SOD level >38.16 U/ml had higher rates of SAI incidence (χ2 = 9.688, p = 0.002; log rank test). Furthermore, Cox regression analysis indicated that a serum SOD level >38.16 U/ml was an independent risk factor for SAI (hazard ratio = 5.836; 95% CI, 1.298-26.244; p = 0.021).Conclusions: Acute-phase serum SOD level could be a predictor of SAI.


Assuntos
Isquemia Encefálica/sangue , Infecções/sangue , Infecções/diagnóstico , Inflamação/sangue , Estresse Oxidativo , Acidente Vascular Cerebral/sangue , Superóxido Dismutase/sangue , Idoso , Biomarcadores/sangue , Isquemia Encefálica/complicações , Feminino , Humanos , Infecções/etiologia , Inflamação/etiologia , Masculino , Malondialdeído/sangue , Pessoa de Meia-Idade , Estresse Oxidativo/fisiologia , Estudos Prospectivos , Sensibilidade e Especificidade , Acidente Vascular Cerebral/complicações
11.
Neuroimmunomodulation ; 26(5): 234-238, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31661704

RESUMO

OBJECTIVE: To explore the diversity and clinical features of anti-glutamate decarboxylase (GAD) antibody-associated neurological diseases. METHODS: Clinical data of a series of 5 patients positive for anti-GAD antibodies were retrospectively analyzed. RESULTS: All 5 patients were female, with a median age of 41.5 years (range 19-60 years). Their neurological symptoms included stiff-person syndrome (SPS), encephalitis, myelitis, cramp, visual loss, and paresthesia. Three patients (60%) were diagnosed with tumors, 2 cases of thymic tumor and 1 of breast cancer. On immunohistochemistry for tumor pathology, expression of GAD65 was found only in 1 patient. Four patients (80%) had abnormal brain MRI findings. All patients received immunotherapy and improved significantly after treatment, but 4 (80%) then experienced a relapse. CONCLUSIONS: Neurological manifestations in anti-GAD-positive patients are diverse and include SPS, encephalitis, myelitis, cramp, visual loss, and paresthesia.


Assuntos
Autoanticorpos/imunologia , Doenças Autoimunes do Sistema Nervoso/fisiopatologia , Glutamato Descarboxilase/imunologia , Síndromes Paraneoplásicas do Sistema Nervoso/fisiopatologia , Adulto , Doenças Autoimunes do Sistema Nervoso/diagnóstico por imagem , Doenças Autoimunes do Sistema Nervoso/imunologia , Encéfalo/diagnóstico por imagem , Neoplasias da Mama/metabolismo , Encefalite/diagnóstico por imagem , Encefalite/imunologia , Encefalite/fisiopatologia , Feminino , Glutamato Descarboxilase/metabolismo , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Cãibra Muscular/imunologia , Cãibra Muscular/fisiopatologia , Mielite/imunologia , Mielite/fisiopatologia , Síndromes Paraneoplásicas do Sistema Nervoso/diagnóstico por imagem , Síndromes Paraneoplásicas do Sistema Nervoso/imunologia , Parestesia/imunologia , Parestesia/fisiopatologia , Recidiva , Estudos Retrospectivos , Rigidez Muscular Espasmódica/imunologia , Rigidez Muscular Espasmódica/fisiopatologia , Neoplasias do Timo/metabolismo , Transtornos da Visão/imunologia , Transtornos da Visão/fisiopatologia , Adulto Jovem
12.
Am J Transl Res ; 11(9): 5673-5688, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31632539

RESUMO

Multiple sclerosis (MS), one of the autoimmune and inflammatory diseases, is a major cause of neurological disability worldwide. The existing clinical treatments are not curable, and better treatments are urgently needed. Mesenchymal stromal cells (MSCs) have shown promise for treating MS, but the favorable effects and mechanism of MSC therapy on MS are still not fully understood. In this study, we analyzed the phenotypic feature of peripheral blood mononuclear cells (PBMCs) in MS patients and found that the patients exhibited an increase in the frequency of B cells, but a markedly decrease in frequency of CD5+ and IL-10+ B cells compared to healthy controls. Infusion of MSCs exhibited a significant therapeutic effect on the experimental autoimmune encephalomyelitis (EAE) mice, infiltration of mononuclear cells and demyelination of the spinal cords were both reduced in CNS of the mice, the frequency of CD5+ IL-10+ B cells in the mice was significantly increased. Additionally, when PBMCs or B cells from MS patients were co-cultured with MSCs, the frequency of CD5+ IL-10+ B cells also increased, the proliferative and immunosuppressive capacity of CD5+ B cells were significantly enhanced while the apoptosis ratio of this cellular subset significantly decreased. Moreover, those effects could be eliminated while the indoleamine 2,3-dioxygenase (IDO) inhibitor, D/L-1MT, was added to the co-cultured cells. In summary, this study suggests that MSCs can control EAE via IDO pathway to promote the proportion and function of CD5+ IL-10+ B cells, providing a promise to treat patients with MS in the clinical setting.

13.
Int J Neurosci ; 129(12): 1183-1188, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31327295

RESUMO

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.


Assuntos
Astrócitos/imunologia , Neoplasias Encefálicas/imunologia , Proteína Glial Fibrilar Ácida/imunologia , Adulto , Idoso , Autoanticorpos/sangue , Autoanticorpos/líquido cefalorraquidiano , Neoplasias Encefálicas/sangue , Neoplasias Encefálicas/líquido cefalorraquidiano , Feminino , Proteína Glial Fibrilar Ácida/sangue , Proteína Glial Fibrilar Ácida/líquido cefalorraquidiano , Humanos , Masculino , Pessoa de Meia-Idade
14.
Ann Clin Transl Neurol ; 6(2): 392-396, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30847372

RESUMO

Our objective was to examine the brain biopsies by histopathology and investigate the prognosis of patients with myelin oligodendrocyte glycoprotein antibody-associated demyelinating pseudotumor. The clinical, MRI, and histological features of two patients with myelin oligodendrocyte glycoprotein antibody-associated demyelinating pseudotumor were reviewed. Both patients were treated with steroid plus rituximab and followed up. The brain biopsies of both cases revealed T cells, macrophages, and complement-mediated demyelination, which was in accord with multiple sclerosis-like pathology. Moreover, both cases showed favorable response to steroid plus rituximab therapy. Our cases add a new variant to the myelin oligodendrocyte glycoprotein-encephalomyelitis spectrum, which favorably responds to immunotherapy.


Assuntos
Autoanticorpos/efeitos adversos , Doenças Desmielinizantes/induzido quimicamente , Glicoproteína Mielina-Oligodendrócito/metabolismo , Malformações do Sistema Nervoso/patologia , Doenças Desmielinizantes/diagnóstico , Doenças Desmielinizantes/patologia , Encefalomielite/tratamento farmacológico , Encefalomielite/patologia , Humanos , Fatores Imunológicos/metabolismo , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/tratamento farmacológico , Esclerose Múltipla/patologia , Glicoproteína Associada a Mielina/metabolismo , Malformações do Sistema Nervoso/tratamento farmacológico , Oligodendroglia/efeitos dos fármacos , Oligodendroglia/metabolismo
15.
Mult Scler Relat Disord ; 29: 94-99, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30690341

RESUMO

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.


Assuntos
Aquaporina 4/líquido cefalorraquidiano , Astrócitos/patologia , Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/líquido cefalorraquidiano , Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/patologia , Encefalite/líquido cefalorraquidiano , Encefalite/patologia , Proteína Glial Fibrilar Ácida/líquido cefalorraquidiano , Glicoproteína Mielina-Oligodendrócito/líquido cefalorraquidiano , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Aquaporina 4/imunologia , Biomarcadores/líquido cefalorraquidiano , Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/fisiopatologia , Encefalite/fisiopatologia , Feminino , Proteína Glial Fibrilar Ácida/imunologia , Humanos , Masculino , Pessoa de Meia-Idade , Glicoproteína Mielina-Oligodendrócito/imunologia , Adulto Jovem
16.
Mult Scler Relat Disord ; 28: 167-171, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30605794

RESUMO

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.


Assuntos
Doenças Autoimunes do Sistema Nervoso/diagnóstico por imagem , Doenças Autoimunes do Sistema Nervoso/imunologia , Meningoencefalite/diagnóstico por imagem , Meningoencefalite/imunologia , Adulto , Anti-Inflamatórios/uso terapêutico , Doenças Autoimunes do Sistema Nervoso/tratamento farmacológico , Doenças Autoimunes do Sistema Nervoso/patologia , Biomarcadores/sangue , Biomarcadores/líquido cefalorraquidiano , Diagnóstico Diferencial , Feminino , Humanos , Inflamação/diagnóstico por imagem , Inflamação/tratamento farmacológico , Inflamação/imunologia , Inflamação/patologia , Meningoencefalite/tratamento farmacológico , Meningoencefalite/patologia , Metilprednisolona/uso terapêutico , Síndrome
17.
Mult Scler Relat Disord ; 28: 177-183, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30611927

RESUMO

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.


Assuntos
Autoanticorpos/líquido cefalorraquidiano , Doenças Autoimunes do Sistema Nervoso/líquido cefalorraquidiano , Bioensaio , Imunofluorescência , Imunoglobulina G/líquido cefalorraquidiano , Animais , Bioensaio/métodos , Biomarcadores/líquido cefalorraquidiano , Cerebelo/imunologia , Imunofluorescência/métodos , Hipocampo/imunologia , Humanos , Estudos Prospectivos , Ratos , Estudos Retrospectivos , Técnicas de Cultura de Tecidos
18.
Front Immunol ; 9: 2802, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30568655

RESUMO

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an autoimmune disease of the nervous system first defined in 2016. GFAP autoantibody, especially IgG that binds to GFAPα, has been reported in the cerebrospinal fluid (CSF) and serum of patients with GFAP astrocytopathy. The positive predictive value of GFAP antibody in the CSF is higher than in the serum. Tissue-based assay (TBA) and cell-based assay (CBA) are both recommended methods for the detection of GFAP antibody. GFAP astrocytopathy is accompanied by neoplasms, but the relationship between virus infection and GFAP astrocytopathy is unclear. GFAP antibody itself does not induce pathological changes; it is only a biomarker for the process of immune inflammation. The pathology of GFAP astrocytopathy in humans is heterogeneous. GFAP astrocytopathy is commonly diagnosed in individuals over 40 years old and most patients have an acute or subacute onset. Clinical manifestations include fever, headache, encephalopathy, involuntary movement, myelitis, and abnormal vision. Lesions involve the subcortical white matter, basal ganglia, hypothalamus, brainstem, cerebellum, and spinal cord. The characteristic MRI feature is brain linear perivascular radial gadolinium enhancement in the white matter perpendicular to the ventricle. Currently, there are no uniform diagnostic criteria or consensus for GFAP astrocytopathy and coexisting neural autoantibodies detected in the same patient make the diagnosis difficult. A standard treatment regimen is yet to be developed. Most GFAP astrocytopathy patients respond well to steroid therapy although some patients are prone to relapse or even die.


Assuntos
Astrócitos , Autoanticorpos , Doenças Autoimunes do Sistema Nervoso , Encéfalo , Proteína Glial Fibrilar Ácida/imunologia , Medula Espinal , Adulto , Idoso , Animais , Astrócitos/imunologia , Astrócitos/metabolismo , Astrócitos/patologia , Autoanticorpos/imunologia , Autoanticorpos/metabolismo , Doenças Autoimunes do Sistema Nervoso/diagnóstico , Doenças Autoimunes do Sistema Nervoso/imunologia , Doenças Autoimunes do Sistema Nervoso/patologia , Doenças Autoimunes do Sistema Nervoso/terapia , Encéfalo/imunologia , Encéfalo/metabolismo , Encéfalo/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Medula Espinal/imunologia , Medula Espinal/metabolismo , Medula Espinal/patologia
19.
Front Immunol ; 9: 2066, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30258442

RESUMO

Objective: To evaluate the efficacy and safety of low-dose mycophenolate mofetil (MMF, 1,000 mg/day) treatment of neuromyelitis optica spectrum disorders (NMOSDs). Methods: This study was a multicenter, open, prospective, follow-up clinical trial. The data include retrospective clinical data from the pretreatment phase and prospective data from the post-treatment phase. From September 2014 to February 2017, NMOSD patients seropositive for aquaporin 4-IgG (AQP4-IgG) were treated with low-dose MMF. Results: Ninety NMOSD patients were treated with MMF for a median duration of 18 months (range 6-40 months). The median annual recurrence rate (ARR) decreased from 1.02 before treatment to 0 (P < 0.0001) after treatment, and the Expanded Disability Status Scale (EDSS) score decreased from 4 to 3 (P < 0.0001). The EDSS score was significantly lower (P = 0.038) after the first 90 days of treatment. The serum AQP4-IgG titer decreased in 50 cases (63%). The median Simple McGill pain score (SF-MPQ) was reduced in 65 patients (88%) with myelitis from 17 (range 0-35) to 11 (range 0-34) after treatment (P < 0.0001). The median Hauser walking index (Hauser Walk Rating Scale) was reduced from 2 (range 1-9) before treatment to 1 (range 0-7) after treatment (P < 0.0001). Adverse events were documented in 43% of the patients, and eight patients discontinued MMF due to intolerable adverse events. Fourteen (16%) of the total patients discontinued MMF after our last follow-up for various reasons and switched to azathioprine or rituximab. Conclusion: Low-dose MMF reduced clinical relapse and disability in NMOSD patients in South China. However, some patients still suffered from adverse events at this dosage. CLINICAL TRIAL REGISTRATION: www.ClinicalTrials.gov, identifier : NCT02809079.


Assuntos
Aquaporina 4/imunologia , Autoanticorpos/imunologia , Ácido Micofenólico , Neuromielite Óptica , Adolescente , Adulto , Idoso , Azatioprina/administração & dosagem , Azatioprina/efeitos adversos , Criança , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Ácido Micofenólico/administração & dosagem , Ácido Micofenólico/efeitos adversos , Neuromielite Óptica/tratamento farmacológico , Neuromielite Óptica/imunologia , Estudos Prospectivos , Rituximab/administração & dosagem , Rituximab/efeitos adversos
20.
Neuroimmunomodulation ; 25(2): 68-72, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30048975

RESUMO

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.


Assuntos
Autoanticorpos/sangue , Glutamato Descarboxilase/sangue , Mielite/sangue , Mielite/diagnóstico , Autoanticorpos/imunologia , Feminino , Glutamato Descarboxilase/imunologia , Humanos , Mielite/imunologia , Estudos Retrospectivos
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