scholarly journals Serum GFAP and NfL as disease severity and prognostic biomarkers in patients with aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder

2021 ◽  
Vol 18 (1) ◽  
Author(s):  
Patrick Schindler ◽  
Ulrike Grittner ◽  
Johanna Oechtering ◽  
David Leppert ◽  
Nadja Siebert ◽  
...  
Keyword(s):  
Disease Severity ◽  
Neuromyelitis Optica ◽  
Single Molecule ◽  
Effect Size ◽  
Clinical Remission ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Prognostic Biomarkers ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Neurofilament Light

Abstract Background Neuromyelitis optica spectrum disorder (NMOSD) is a frequently disabling neuroinflammatory syndrome with a relapsing course. Blood-based disease severity and prognostic biomarkers for NMOSD are a yet unmet clinical need. Here, we evaluated serum glial fibrillary acidic protein (sGFAP) and neurofilament light (sNfL) as disease severity and prognostic biomarkers in patients with aquaporin-4 immunoglobulin (Ig)G positive (AQP4-IgG+) NMOSD. Methods sGFAP and sNfL were determined by single-molecule array technology in a prospective cohort of 33 AQP4-IgG+ patients with NMOSD, 32 of which were in clinical remission at study baseline. Sixteen myelin oligodendrocyte glycoprotein IgG-positive (MOG-IgG+) patients and 38 healthy persons were included as controls. Attacks were recorded in all AQP4-IgG+ patients over a median observation period of 4.25 years. Results In patients with AQP4-IgG+ NMOSD, median sGFAP (109.2 pg/ml) was non-significantly higher than in MOG-IgG+ patients (81.1 pg/ml; p = 0.83) and healthy controls (67.7 pg/ml; p = 0.07); sNfL did not substantially differ between groups. Yet, in AQP4-IgG+, but not MOG-IgG+ patients, higher sGFAP was associated with worse clinical disability scores, including the Expanded Disability Status Scale (EDSS, standardized effect size = 1.30, p = 0.007) and Multiple Sclerosis Functional Composite (MSFC, standardized effect size = − 1.28, p = 0.01). While in AQP4-IgG+, but not MOG-IgG+ patients, baseline sGFAP and sNfL were positively associated (standardized effect size = 2.24, p = 0.001), higher sNfL was only non-significantly associated with worse EDSS (standardized effect size = 1.09, p = 0.15) and MSFC (standardized effect size = − 1.75, p = 0.06) in patients with AQP4-IgG+ NMOSD. Patients with AQP4-IgG+ NMOSD with sGFAP > 90 pg/ml at baseline had a shorter time to a future attack than those with sGFAP ≤ 90 pg/ml (adjusted hazard ratio [95% confidence interval] = 11.6 [1.3–105.6], p = 0.03). In contrast, baseline sNfL levels above the 75th age adjusted percentile were not associated with a shorter time to a future attack in patients with AQP4-IgG+ NMOSD. Conclusion These findings suggest a potential role for sGFAP as biomarker for disease severity and future disease activity in patients with AQP4-IgG+ NMOSD in phases of clinical remission.

scholarly journals Aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder with recurrent short partial transverse myelitis and favorable prognosis: Two new cases

2017 ◽  
Vol 23 (14) ◽  
pp. 1950-1954 ◽  
Author(s):  
Jinhua Zhang ◽  
Fang Liu ◽  
Yiqi Wang ◽  
Ying Yang ◽  
Yuehong Huang ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Acute Attack ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Attack Phase ◽  
Long Term Prognosis

Understanding the characteristics of neuromyelitis optica spectrum disorder (NMOSD) with recurrent short partial transverse myelitis (SPTM), which is very rare, contributes to the differential diagnosis of multiple sclerosis (MS). We present two Chinese aquaporin-4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD cases who had at least twice SPTM during 4 and 6 years of follow-up, respectively. Their SPTMs have been mild and responded well to corticosteroids just like in the case of MS. The findings highlight the need of searching for serum AQP4-IgG (cell-based assay strongly recommended) in patients with recurrent SPTM and suggest that those patients may have a mild acute attack phase and favorable long-term prognosis.

Cortical oscillopsia without nystagmus, an isolated symptom of neuromyelitis optica spectrum disorder with anti-aquaporin 4 antibody

2011 ◽  
Vol 18 (2) ◽  
pp. 244-247 ◽  
Author(s):  
Sung-Min Kim ◽  
Ji-Soo Kim ◽  
Young Eun Heo ◽  
Hye-Ran Yang ◽  
Kyung Seok Park
Keyword(s):  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Ocular Manifestation ◽  
Initial Manifestation ◽  
Ocular Symptoms ◽  
Head Tremor ◽  
Aquaporin 4 Antibody

Neuromyelitis optica (NMO), mainly affecting optic nerve and spinal cord, can also manifest diverse ocular symptoms due to brain abnormalities. We present a cortical oscillopsia without nystagmus or head tremor in a patient with neuromyelitis optica spectrum disorder (NMOSD) with anti-aquaporin 4 antibody. This rare ocular manifestation, which is easily underestimated owing to absence of the typical nystagmus, can be an initial manifestation of NMOSD.

scholarly journals Investigating the Presence of Interattack Astrocyte Damage in Neuromyelitis Optica Spectrum Disorder

2021 ◽  
Vol 8 (3) ◽  
pp. e965 ◽  
Author(s):  
Jae-Won Hyun ◽  
Yeseul Kim ◽  
So Yeon Kim ◽  
Min Young Lee ◽  
Su-Hyun Kim ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Single Molecule ◽  
Spectrum Disorder ◽  
Cancer Center ◽  
Multiple Time ◽  
Healthy Controls ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Serum Samples ◽  
Neurofilament Light Chain ◽  
Cutoff Value

ObjectivesInformation on subclinical astrocyte damage can provide further insight into neuromyelitis optica spectrum disorder (NMOSD) pathophysiology and disease-monitoring strategies. To investigate whether astrocyte and neuroaxonal damage occurs during interattack periods in individuals with NMOSD through longitudinal measurement of serum glial fibrillary acidic protein (sGFAP) and neurofilament light chain (sNfL) at multiple time points.MethodssGFAP and sNfL levels were measured in 187 serum samples from 20 participants with NMOSD treated with rituximab (median follow-up: 24 months) and 19 age-/sex-matched healthy controls using a highly sensitive single-molecule array assay. From the NMOSD cohort of National Cancer Center, Korea, 14 clinically stable participants were randomly selected for focused investigation of interattack periods, and 6 participants with clinical attacks despite treatment were enrolled for attack-related measurements.ResultsSignificant elevations of sGFAP levels were observed in all clinical attacks, and 95% (19/20) of patients showed reduction of sGFAP levels below the cutoff value (3 SDs above mean levels in age-/sex-matched healthy controls) within 3 months of their clinical attacks. The sGFAP levels were consistently low during interattack periods in 90% (17/19) of patients whose sGFAP levels returned to below the cutoff value. Changes in sNfL levels were similar to but slower than those in sGFAP levels.ConclusionsSubclinical astrocyte damage represented by increasing sGFAP levels rarely occurred during interattack periods in individuals with NMOSD; however, a certain degree of astrocyte damage did occur at the time of clinical attacks without exception, but it was not evident within 3 months of the attack.

scholarly journals Anti-myelin Oligodendrocyte Glycoprotein in Aquaporin-4 Negative Neuromyelitis Optica Spectrum Disorder

2021 ◽  
Vol 7 (1) ◽  
pp. 10-16
Author(s):  
Vahid Shaygannejad ◽  
◽  
Mahdi Barzegar ◽  
Navid Manouchehri ◽  
Nafiseh Esmaeil ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Cross Sectional Study ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
University Hospital ◽  
Control Group ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Cross Sectional ◽  
Healthy Control

Background: The absence of Aquaporin-4 Antibody (AQP4-Ab) in a fraction of the Neuromyelitis Optica Spectrum Disorder (NMOSD) patients has led to a search for other serologic markers. Myelin Oligodendrocyte Glycoprotein (MOG) is a protein component of the myelin sheets encapsulating the neural fibers. Objectives: We aimed to compare the presence and levels of anti-MOG (Ig-G) in a group of seronegative NMOSD patients with a healthy control group. Materials & Methods: In this cross-sectional study, 30 NMOSD patients with negative AQP-Ab status, who were referred to the Neurology Clinic of Kashani University Hospital in Isfahan City, Iran, from March 2015 to March 2016, and 26 healthy controls were consecutively recruited. Their baseline demographic and clinical data were recorded. Serum anti-MOG levels were measured in both groups. The obtained data were analyzed using the Student t-test, Mann-Whitney U, and Chisquare test in SPSS V. 18. Results: The anti-MOG test results were statistically higher in patients (n=12, 37.5%) compared to controls (n=0, 0%) (P<0.0001). The level of anti-MOG in Healthy Control (HC) was higher compared to patients with negative anti-MOG (P<0.0001) and was lower than patients with positive anti-MOG (P<0.0001). Conclusion: Our study showed that nearly one-third of seronegative NMOSD patients were positive for MOG-Ab. Further studies are needed to assess the characteristics and outcome of these patients.

Anti‐aquaporin‐4 antibody‐positive neuromyelitis optica spectrum disorder associated with central nervous system tuberculosis

2019 ◽  
Vol 11 (1) ◽  
pp. 40-44
Author(s):  
Enrique Gomez‐Figueroa ◽  
Christian Garcia‐Estrada ◽  
Adriana Casallas-Vanegas ◽  
Indhira Zabala-Angeles ◽  
Ramon Lopez-Martinez ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Neuromyelitis Optica ◽  
Spectrum Disorder ◽  
Aquaporin 4 ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Aquaporin 4 Antibody
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