Clinical characteristics of 14 patients with autoimmune glial fibrillary acidic protein astrocytosis with central nervous system inflammation as the main manifestation / 中风与神经疾病杂志

@#Objective To explore the clinical characteristics of patients with autoimmune glial fibrillary acidic protein(GFAP) astrocytosis with central nervous system inflammation as the main manifestation.Methods Twenty-one patients with autoimmune GFAP astrocytosis with positive cerebrospinal fluid GFAP antibody were collected from January 2017 to April 2020.The clinical data of 14 patients with cerebrospinal fluid GFAP antibody positive were retrospectively analyzed.Results Among the 14 patients,4 were female and 10 were male.The age of onset was(42±16) years old.The main clinical manifestations of the patients were headache(13/14),fever(13/14),disturbance of consciousness(11/14),mental symptoms(10/14),epilepsy(4/10),defecation disorder(8/14),hypoxemia(6/14),limb paralysis(2/14),etc.MRI showed abnormal enhancement of intracranial meninges in 13 cases(13/14) and spinal cord involvement in 5 cases(5/14).Conclusion There are more males than females with autoimmune GFAP astrocytosis with cerebrospinal meningitis and other central system inflammation.MRI manifestations are complex.Intracranial and spinal cord can be involved at the same time.Intracranial lesions mainly involve meninges,which can be combined with cerebral cortex damage.In myelopathy,both the spinal cord and the spinal cord are involved,and the spinal cord membrane is mainly involved.

Magnetic resonance imaging of the spinal cord and clinical characteristics in patients with autoimmune glial fibrillary acidic protein astrocytopathy / 中华神经科杂志

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.

The clinical and radiological features of MOG antibody positive demyelinating diseases of central nervous system / 中国神经精神疾病杂志

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.

Associations of aquaporin-4 promoter polymorphism to multiple sclerosis and neuromyelitis optica in southern Chinese population / 中华神经科杂志

Objective To investigate the associations of aquaporin-4 (AQP4) promoter polymorphisms with anti-AQP4 antibody and genetic susceptibility to multiple sclerosis (MS) and neuromyelitis optica (NMO) in Southern Chinese population.Methods The polymorphisms of AQP4promoter 0 and 1 were analyzed by PCR and DNA sequencing in 18 NMO,38 MS,13 recurrent myelitis (RM),6 recurrent optic neuritis (RON)patients and 39 healthy controls. Results Fourteen polymorphism loci were observed in AQP4-promoter 0,while 6 ones were observed in AQP4-promoter 1.Among them,the incidence rate of polymorphism at position - 1003 bp (A-G) of AQP4-promoter 0 in anti-AQP4 antibody-positive patients was significantly higher than that in anti-AQP4 antibody-negative patients and controls (former:13/18 vs 20/45,P =0.046; latter:13/18 vs 10/39,P =0.001 ).The incidence rates of polymorphism at position between -401 bp and -400 bp ( C inserted) of AQP4-promoter 1 in anti-AQP4 antibody-positive and -negative patients were significantly higher than that in controls( former:5/16 vs 0/28,P =0.008; latter:8/38 vs 0/28,P =0.027 ). The incidence rates of polymorphism at position - 1003 bp (A-G) of AQP4-promoter 0 and position between -401 bp and -400 bp ( C inserted)of AQP4-promoter 1 in patients with NMO and MS were significantly higher than that in controls( NMO:11/18 vs 10/39,P =0.010;4/15 vs 0/28,P =0.020; MS:19/38 vs 10/39,P =0.027;8/34 vs 0/28,P =0.018).Conclusions Polymorphisms loci were observed in AQP4-promoter 0 and AQP4-promoter 1,which may have an influence on the susceptibility to MS and NMO.Polymorphism at position - 1003 bp ( A-G) of AQP4-promoter 0 may be related to the emergence of anti-AQP4 antibody in patients with NMO and MS.

Detection of aquaporins-4:methods comparison and clinical significance for the diagnosis of neuromyelitis optica / 中华神经科杂志

Objective To compare the efficiency of original neuromyelitis optica(NMO)-IgG assay of detecting NMO-IgG with a new anti-aquaporin-4(AQP4)assay of detecting AQP4,and to explore the accuracy of the method in the diagnosis of NMO and multiple sclerosis(MS).Methods The sera were obtained from 44 patients with NMO and 46 patients with MS and were tested by both NMO-IgG and antiAOP4 assays.NMO-IgG was identified by original NMO-IgG assay with a substrate from mouse brain.AntiAQIP4 was detected by anti-AQP4 antibody assay.The results from the two assays were statistically analyzed to compare accuracy and specificity of the methods.Results The results of the two assays were concordant in 45 testing negative cases and 36 positive cases(Kappa=0.798.P=0.000).The McNemar test showed that the positive rate of the two assays were not significantly different(P=1.000).The NMO-IgG assay showed 77.3% sensitivity,87% specificity,82.2% diagnosis accuracy,85%positive predictive value,87% negative predictive value.and 74.3%Younden index. The anti-AOP4 antibody assay showed 88.6% sensitivity,95.7%specificity,92.2% diagnosis accuracy,98.1% positive predictive value,89.8% negative predictive value.and 84.3% Younden index.Conclusions This study demonstrated that NMO-IgG and AQP4 antibody detection have high sensitivity and specificity to detect NMO and MS.Anti-AQP4 detected by anti-AQP4 antibody assay may be more useful for NMO diagnosis.

Tat-NBD peptide inhibits inflammation effects related to NF-kB on AR42J cell / 中华急诊医学杂志

Objective To investigate the effects of Tat-NBD(NEMO-binding domain,NBD)peptide on LPS-stimulated AR42J acinus cells inflammatory response.Method Lipopolysaccharide(LPS)was added to culture media at doses of 10 mg/kg for 24 hours to stimulate the AR42J cells.For pretreatment.cells were incubated with different peptides for 2 hours before LPS stimulation.The expression of TNF-α mRNA WaS conducted using a semi-quantitative RT-PCR method.TNF-α protein in culture medium were detected by enzyme linked immunosorbent assay(ELISA).The expression and translocation of the NF-kB-p65 protein of AR42J was determined by Strept Actividin-Biotin Complex(SABC)method.Results LPS(10 mg/L)resulted in an increase of TNF-αmRNA and TNF-αprotein,whereas significant inhibitory effects were observed when cells were incubated with Tat-NBD(WT)just on dose of 0.1 me/L(P＜0.05).The Tat-NBD(WT)peptide decreased inflammatory cytokine expression by a dose-dependent manner and its peak role was on dose of 100 mg/L.Consisting with TNF-α expression decrease,NF-kB-p65 expression signitieantly decreased in Tat-NBD(WT)pretreatment group,especially on the largest dose.NO significant changes in the control peptide group.Conclusions Tat-NBD(WT)peptide can inhibit the inflammation of acinus simulated by LPS.

Neuromyelitis optica-IgG detection in serum by indirect immunofluorescence assay / 中华神经科杂志

Objective To establish a method to detect neuromyelitis optiea (NMO)-IgG in patients serum using indirect immunofluorescence assay (IFA). Methods The normal tissues (cerebellum/ midbrain, kidney and stomach) from C57 mice were cryosectioned onto microscope slides as detective substrate. For NMO-IgG detection, isolated serum from patient with NMO, multiple sclerosis (MS), optic neuritis or myelitis was incubated with the tissue sections on the slide at 4℃ overnight and subsequently incubated with a fluorochrome-cojugated lgG specific for human. For double immunostaining with aquaporius-4 (AQP4), the slides were incubated with primary antibody of AQP4 and secondary antibody of IgG-TRITC. Detection of NMO-IgG and its co-localization with AQP4 was analyzed using fluorescence microscope. Results All 182 serum samples from patients were tested using IFA. Some samples revealed a characteristic immunohistochemical staining of NMO-IgG in mouse CNS tissues, predominately in pia and subpia, and capillaries in white and grey matter in the cerebellum, midbrain, and spinal cord. Double immunostaining with AQP4 demonstrated the co-localization of NMO-IgG with AQP4. Conclusions We established an IFA using a substrate from C57 mouse cerebellum/midbrain, kidney and stomach tissue to detect NMO-IgG in patient serum. This method is specific and efficient in detection and may be useful in diagnosis and differential diagnosis of neuromyelitis optica.

Effects of NF-κB essential modifier binding domain peptides on rats with acute necrotizing pancreatitis / 中华胰腺病杂志

n of IL-6 and TNF-alpha,alleviate the inflammation of ANP.

The more understanding,the more facility to diagnosis and therapy of multiple sclerosis / 中国实用内科杂志

The diagnostic core of multiple sclerosis(MS) always requires dissemination in space and time since the publication of the first criteria over 40 years ago.Though several modified clinical criterias have been proposed to diagnosis MS,no paitents must meet such condition in diagnostic criteria because of the reticular clinical manifestation of this disease.The diagnosis of MS is still based on clinical parameters including detailed history and a careful examination to exclude alternative disease.The effects of some disease modifying agents to MS have been confirmed by evidence-based medicine.But the intact treatment plan must contain aspects of acute attacks,prevention of relapses and progression,management of symptoms,and rehabilitation.The individualized treatment is needed according to the medical insurance,payment ability and other influencing factors in China.

Activation of NF-?B and anti-inflammation mechanism of pyrrolidine dithiocarb amate in rats with acute colitis / 中国临床药理学与治疗学

AIM: To investigate DNA-binding activity of nuclear factor ka ppa B (NF-?B) and pyrrolidine dithiocarbamate (PDTC) anti-inflammation effect and mechanism in trinitrobenzene sulfonic acid (TNBS)-induced acute colitis in rats. METHODS: Acute colitis was induced by instilling TNBS/ ethanol into the lumen of the rat colon. Rats were randomized into PDTC-treat g roup (including group P10,P25,P50,P100), TNBS/ethanol group and normal control g roup. The rats in PDTC-treated group were injected intraperitoneally with PDTC at the dosages of 10, 25, 50, and 100 mg?kg -1 , respectively. Rats wer e killed at 4 h after enema. Colonic inflammation, the ratio of wet/dry weig ht and MPO, SOD, and MDA activity were detected. DNA-binding activity of NF-? B was assessed by EMSA. RESULTS: NF-?B activity in colon homog enate was increased markedly at acute colitis in rats. PDTC attenuates the devel opment of rat colonic inflammation but not by reducing the NF-?B activity. CONCLUSION: NF-?B is activated in rats with acute colitis, which may be a mechanism of self-protection. PDTC can attenuate the development of in flammation.