aqp4 antibody
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2021 ◽  
pp. 135245852110603
Author(s):  
Jae-Won Hyun ◽  
Hye Lim Lee ◽  
Jaehong Park ◽  
Jiah Kim ◽  
Ju-Hong Min ◽  
...  

In a large acute myelitis cohort, we aimed to determine whether brighter spotty lesions (BSLs)—using the refined terminology—on spinal magnetic resonance imaging (MRI) help distinguish aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD) from myelin oligodendrocyte glycoprotein antibody disease (MOGAD). An experienced neuro-radiologist and two neurologists independently analyzed 133 spinal MRI scans (65 from MOGAD and 68 from AQP4-NMOSD) acquired within 1 month of attacks. BSLs were observed in 18 of 61 (30%) participants with AQP4-NMOSD, while none of 49 participants with MOGAD showed BSL ( p < 0.001). BSL during the acute phase would be useful to differentiate AQP4-NMOSD from MOGAD.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Sitong Guo ◽  
Hanqiu Jiang ◽  
Libin Jiang ◽  
Jingting Peng ◽  
Hongjuan Liu ◽  
...  

AbstractThis study investigated the factors influencing intravenous methylprednisolone pulse (IVMP) therapy for recovering visual acuity in Chinese patients with aquaporin-4 (AQP4) antibody-seropositive neuromyelitis optica-related optic neuritis (NMO-ON). This retrospective case series included 243 affected eyes of 182 patients (36 male, 146 female) diagnosed with NMO-ON in the Neuro-Ophthalmology Clinic of Beijing Tongren Hospital from September 2012 to September 2020. All patients with AQP4-antibody seropositivity had clinical manifestations of acute ON, excluding other diagnoses and received IVMP treatment at 500 mg/day or 1000 mg/day for 3 days. Primary outcome was the extent of improvement in logMAR visual acuity after IVMP treatment. The therapeutic influences of sex, age, baseline visual acuity, therapeutic intervals, and IVMP dose on acute NMO-ON were analysed. Chi-square tests, Mann–Whitney U-tests, Kruskal–Wallis tests, Spearman’s correlation coefficients, and multiple linear regression were used for statistical analysis. Age ranged between 7 and 80 years (median age, 44; interquartile range [IQR], 29–52) years. Among the 243 eyes, the median improvement in logMAR visual acuity was 0.3 (IQR, 0–0.9). Therapeutic efficacy of IVMP was significantly higher in female than in male patients (Z = 2.117, P = 0.034). The treatment effect gradually decreased with increase in age at onset (Rs = 0.157, P = 0.015), and visual improvement was significantly lower in patients aged > 50 years than in those ≤ 50 years (Z = 2.571, P = 0.010). When patients had low visual acuity at onset, improvements were more obvious (rho =  − 0.317, P < 0.001); however, final visual acuity was still low (rho = 0.688, P < 0.001). Therapeutic effect was negatively correlated with therapeutic intervals (rho = 0.228, P = 0.001). Dosage of methylprednisolone (1000 mg/day or 500 mg/day) did not significantly influence treatment efficacy (Z = 0.951 P = 0.342). Therefore, IVMP therapy can improve visual acuity in the affected eyes of patients with AQP4 antibody-seropositive NMO-ON with similar effect at 500 mg/day and 1000 mg/day doses. Sex, age at onset, and therapeutic intervals may influence the efficacy of IVMP in patients with NMO-ON.


2021 ◽  
Vol 11 (5) ◽  
pp. 341-352
Author(s):  
Mark J Tullman ◽  
Aram Zabeti ◽  
Scott Vuocolo ◽  
Quinn Dinh

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease characterized by recurrent optic neuritis and transverse myelitis often resulting in severe disability. Anti-AQP4-immunoglobulin G (IgG) is a pathogenic product of CD19-positive plasma cells found in most, but not all, individuals with NMOSD and is associated with immune-mediated neurologic injury. Inebilizumab, an afucosylated humanized IgG1κ, anti-CD19 monoclonal antibody, may target pathogenic CD19-expressing B cells. In a Phase II/III trial, inebilizumab significantly reduced the proportion of participants experiencing an NMOSD attack and was well tolerated versus placebo. Fewer treated participants had worsening disability than those receiving placebo. Inebilizumab was approved in 2020 by the US FDA for treatment of anti-AQP4 antibody positive NMOSD.


2021 ◽  
Vol 12 ◽  
Author(s):  
Laura Clarke ◽  
Simon Arnett ◽  
Wajih Bukhari ◽  
Elham Khalilidehkordi ◽  
Sofia Jimenez Sanchez ◽  
...  

Neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) are inflammatory diseases of the CNS. Overlap in the clinical and MRI features of NMOSD and MS means that distinguishing these conditions can be difficult. With the aim of evaluating the diagnostic utility of MRI features in distinguishing NMOSD from MS, we have conducted a cross-sectional analysis of imaging data and developed predictive models to distinguish the two conditions. NMOSD and MS MRI lesions were identified and defined through a literature search. Aquaporin-4 (AQP4) antibody positive NMOSD cases and age- and sex-matched MS cases were collected. MRI of orbits, brain and spine were reported by at least two blinded reviewers. MRI brain or spine was available for 166/168 (99%) of cases. Longitudinally extensive (OR = 203), “bright spotty” (OR = 93.8), whole (axial; OR = 57.8) or gadolinium (Gd) enhancing (OR = 28.6) spinal cord lesions, bilateral (OR = 31.3) or Gd-enhancing (OR = 15.4) optic nerve lesions, and nucleus tractus solitarius (OR = 19.2), periaqueductal (OR = 16.8) or hypothalamic (OR = 7.2) brain lesions were associated with NMOSD. Ovoid (OR = 0.029), Dawson's fingers (OR = 0.031), pyramidal corpus callosum (OR = 0.058), periventricular (OR = 0.136), temporal lobe (OR = 0.137) and T1 black holes (OR = 0.154) brain lesions were associated with MS. A score-based algorithm and a decision tree determined by machine learning accurately predicted more than 85% of both diagnoses using first available imaging alone. We have confirmed NMOSD and MS specific MRI features and combined these in predictive models that can accurately identify more than 85% of cases as either AQP4 seropositive NMOSD or MS.


2021 ◽  
Vol 18 (1) ◽  
Author(s):  
Teruyuki Ishikura ◽  
Makoto Kinoshita ◽  
Mikito Shimizu ◽  
Yoshiaki Yasumizu ◽  
Daisuke Motooka ◽  
...  

Abstract Background Intractable neuropathic pain is a common symptom of neuromyelitis optica spectrum disorder (NMOSD). However, the underlying mechanism of NMOSD pain remains to be elucidated. In this study, we focused on ATP, which is one of the damage-associated molecular patterns, and also a well-recognized molecule involved in peripheral neuropathic pain. Methods We assessed the development of pain symptoms by injecting anti-AQP4 recombinant autoantibodies (rAQP4 IgG) into rat spinal cords. We incubated HEK293 cells expressing AQP4 (HEK-AQP4) and rat astrocytes with rAQP4 IgG and assessed the level of ATP in the supernatant. We performed transcriptome analysis of the spinal cords injected with rAQP4 IgG. Pharmacological inhibition was also applied to investigate the involvement of ATP in the development of neuropathic pain in our rat model. The ATP concentration within the cerebrospinal fluid was examined in patients with NMOSD and other neurological diseases. Results Development of mechanical allodynia was confirmed in rAQP4 IgG–treated rats. AQP4-Ab–mediated extracellular ATP release from astrocytes was observed in vitro, and pharmacological inhibition of ATP receptor reversed mechanical allodynia in the rAQP4 IgG–treated rats. Furthermore, transcriptome analysis revealed elevation of gene expressions related to several ATP receptors including P2rx4 and IL1B in the spinal cord of rAQP4 IgG–treated rats. In patients, CSF ATP concentration was significantly higher in the acute and remission phase of NMOSD than in multiple sclerosis or other neurological disorders. Conclusion Anti-AQP4 antibody was shown to induce the release of extracellular ATP from astrocytes. The ATP-mediated development of mechanical allodynia was also suggested in rats treated with anti-AQP4 antibody. Our study indicates the pivotal role of ATP in the pain mechanism of NMOSD.


Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012599
Author(s):  
Hannah H. Zhao-Fleming ◽  
Cristina Valencia Sanchez ◽  
Elia Sechi ◽  
Jery Inbarasu ◽  
Eelco F. Wijdicks ◽  
...  

Background and Objective:Severe attacks of myelin oligodendrocyte glycoprotein (MOG)-antibody-associated disorder (MOGAD) and aquaporin-4 (AQP4)-antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD) may require ventilatory support but data on episodes is limited, particularly for MOGAD. We sought to compare the frequency, characteristics, and outcomes of MOGAD and AQP4-NMOSD attacks requiring ventilatory support.Methods:This retrospective descriptive study identified Mayo Clinic patients (1/1/1996-12/1/2020) with MOGAD or AQP4-NMOSD and an attack requiring non-invasive or invasive ventilation at Mayo Clinic or an outside facility by searching for relevant terms in their electronic medical record. Inclusion criteria were: 1) Attack-related requirement for non-invasive (BiPAP or CPAP) or invasive respiratory support (mechanical ventilation); 2) MOG or AQP4 antibody positivity with fulfillment of MOGAD and AQP4-NMOSD clinical diagnostic criteria, respectively; 3) Sufficient clinical details. We collected data on demographics, co-morbidities, indication for and duration of respiratory support, MRI findings, treatments, and outcomes. The race of those with attacks requiring respiratory support were compared to those without such attacks in MOGAD and AQP4-NMOSD.Results:Attacks requiring ventilatory support were similarly rare in MOGAD (8/279, 2.9%) and AQP4-NMOSD patients (11/503, 2.2%) (p=0.63). The age at attack (median years [range]) (MOGAD, 31.5[5-47] vs AQP4-NMOSD, 43[14-65]; p=0.01) and percentage of female sex (MOGAD, 3/8[38%] vs AQP4-NMOSD, 10/11[91%]; p=0.04) differed. The reasons for ventilation differed between MOGAD (inability to protect airway from seizure, encephalitis or encephalomyelitis with attacks of: acute disseminated encephalomyelitis, 5[62.5%]; or unilateral cortical encephalitis, 3[37.5%]) and AQP4-NMOSD (inability to protect airway from cervical myelitis, 9[82%]; rhombencephalitis, 1[9%]; or combinations of both, 1[9%]). Median ventilation duration for MOGAD was 2 days (range, 1-7) versus 19 days (range, 6-330) for AQP4-NMOSD (p=0.01). All MOGAD patients recovered, but 2/11 (18%) of AQP4-NMOSD died from the attack. For AQP4-NMOSD, Black race was over-represented with attacks requiring ventilatory support versus those without these episodes (5/11[45%] versus 88/457[19%]; p=0.045).Discussion:Ventilatory support is rarely required for MOGAD and AQP4-NMOSD attacks and the indications differ. When compared to MOGAD, these attacks in AQP4-NMOSD may have higher morbidity and mortality and those of Black race were more predisposed, which we suspect may relate to socially mediated health inequality.


2021 ◽  
Vol 51 ◽  
pp. 102879
Author(s):  
Papp Viktoria ◽  
Kim D.P. Trones ◽  
Melinda Magyari ◽  
Nils Koch-Henriksen ◽  
Anna Iljicsov ◽  
...  

2021 ◽  
pp. 135245852110189
Author(s):  
Silvia Messina ◽  
Romina Mariano ◽  
Adriana Roca-Fernandez ◽  
Ana Cavey ◽  
Maciej Jurynczyk ◽  
...  

Background: Identifying magnetic resonance imaging (MRI) markers in myelin-oligodendrocytes-glycoprotein antibody-associated disease (MOGAD), neuromyelitis optica spectrum disorder-aquaporin-4 positive (NMOSD-AQP4) and multiple sclerosis (MS) is essential for establishing objective outcome measures. Objectives: To quantify imaging patterns of central nervous system (CNS) damage in MOGAD during the remission stage, and to compare it with NMOSD-AQP4 and MS. Methods: 20 MOGAD, 19 NMOSD-AQP4, 18 MS in remission with brain or spinal cord involvement and 18 healthy controls (HC) were recruited. Volumetrics, lesions and cortical lesions, diffusion-imaging measures, were analysed. Results: Deep grey matter volumes were lower in MOGAD ( p = 0.02) and MS ( p = 0.0001), compared to HC and were strongly correlated with current lesion volume (MOGAD R = −0.93, p < 0.001, MS R = −0.65, p = 0.0034). Cortical/juxtacortical lesions were seen in a minority of MOGAD, in a majority of MS and in none of NMOSD-AQP4. Non-lesional tissue fractional anisotropy (FA) was only reduced in MS ( p = 0.01), although focal reductions were noted in NMOSD-AQP4, reflecting mainly optic nerve and corticospinal tract pathways. Conclusion: MOGAD patients are left with grey matter damage, and this may be related to persistent white matter lesions. NMOSD-AQP4 patients showed a relative sparing of deep grey matter volumes, but reduced non-lesional tissue FA. Observations from our study can be used to identify new markers of damage for future multicentre studies.


BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Jung Lung Hsu ◽  
Ming-Feng Liao ◽  
Kuo-Hsuan Chang ◽  
Mei-Yun Cheng ◽  
Long-Sun Ro

Abstract Background Neuromyelitis optica spectrum disorder (NMOSD) is a rare neuroinflammatory disorder of the central nervous system that typically involves the optic nerve, the spinal cord and other specific brain regions. In relapse of the disease, factors associated with clinical features and lesion severity are important for clinicians to predict disease-related disability. Methods We retrospectively analyzed 22 female patients with NMOSD who had spinal cord lesions. Detailed clinical features, onset symptoms, motor disability, relapse episodes, serum aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) autoantibodies and MRI characteristics were documented to correlate their associations with the nadir and three-month Expanded Disability Status Scale (EDSS) scores. Patients with three-month EDSS scores below four (< 4) were categorized as the good outcome group, while those with scores of four or more (> 4) were categorized as the poor outcome group. Results In patients with NMOSD, the mean age was 44.5 ± 12.8 years, and the mean three-month EDSS score was 4.3 ± 1.9. A significantly higher all-limb muscle power score was found in the good EDSS group than in the poor EDSS group (p = 0.01). A tendency toward longer follow-up periods and lower anti-AQP4 antibody levels was found in the good outcome group. Serum anti-AQP4 antibodies were present in 86% of patients with NMOSD, and MOG autoantibodies were found in one anti-AQP4 antibody-negative patient (33.3%). In patients with NMOSD, more than 40% of spinal cord lesions were distributed at the middle cervical and upper thoracic levels. Conclusions Our findings suggest that EDSS scores and MRC scores at the nadir had significant associations with three-month EDSS scores. The topographic distributions of the spinal cord lesions might relate to different serum anti-AQP4 antibody status. However, further studies will be needed to corroborate this finding.


BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Rui Li ◽  
Danli Lu ◽  
Hao Li ◽  
Yuge Wang ◽  
Yaqing Shu ◽  
...  

Abstract Background Early stage neuromyelitis optica spectrum disorders (NMOSD) with non-opticospinal manifestations as initial symptoms are easily misdiagnosed; however, data on the full symptom profile are limited. Moreover, the clinical characteristics and long-term outcomes of these patients remain unknown. We sought to analyze the clinical characteristics, imaging features, and long-term outcomes of NMOSD with non-opticospinal manifestations as initial symptoms. Methods We retrospectively included relevant patients from our center. Clinical, demographic, magnetic resonance imaging, treatment, and outcome data were compared according to the non-opticospinal vs. opticospinal initial symptoms. Results We identified 43 (9.13 %) patients with non-opticospinal initial symptoms among 471 patients with NMOSD. Of these, 88.37 % developed optic neuritis/myelitis during an average follow-up period of 6.33 years. All the non-opticospinal symptoms were brain/brainstem symptoms. Most of the symptoms and associated brain lesions were reversible. These patients had a younger onset age (P < 0.001), lower serum aquaporin-4 (AQP4) antibody titers (P = 0.030), and a lower Expanded Disability Status Scale (EDSS) score at onset (P < 0.001) and follow-up (P = 0.041) than NMOSD patients with opticospinal initial symptoms. In addition, EDSS scores reached 3.0 (indicating moderate disability) later than in patients with opticospinal initial symptoms (P = 0.028). Conclusions Patients with NMOSD with non-opticospinal initial symptoms have a younger onset age, lower serum AQP4 antibody titers, and better clinical outcomes.


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