scholarly journals Neuromyelitis optica spectrum disorders in the Arabian Gulf: challenges and growing experience

2020 ◽  
Vol 6 (1) ◽  
pp. 205521731985019
Author(s):  
Raed Alroughani ◽  
Najeeb Qadi ◽  
Jihad Inshasi ◽  
Eslam Shosha
Keyword(s):  
Neuromyelitis Optica ◽  
Arabian Gulf ◽  
Target Antigen ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Gulf Region ◽  
Presenting Symptoms ◽  
Disease Pattern ◽  
Variable Sensitivity ◽  
History Of ◽  
Spectrum Disorders

Neuromyelitis optica spectrum disorders (NMOSD) have been studied in different ethnic groups, including Asians, African-Americans, and Caucasians. Demonstrating the clinical features among diverse communities is important to understand the variable disease phenotypes, which will lead to further classification and better clinical management. Testing for antibody against aquaporin-4 (AQP4), the most common target antigen in NMOSD, is not available in many countries and tests use different methods, with variable sensitivity. With negative antibody results, the diagnosis of NMOSD becomes challenging and may affect the outcomes of patients with NMOSD. There are no adequate studies that assess NMOSD cohorts in the Arabian Gulf region, despite the increasing number of diagnosed cases. It is worth assessing NMOSD cohorts in the Arabian Gulf population to study the natural history of disease and to establish an epidemiological background for future perspectives. Various challenges to implement such a mission are outlined, including disease rarity, overlapping presenting symptoms and signs, which posed the issue of mimickers in the differential diagnosis, lack of specialized clinics, absence of highly sensitive testing methods for diagnosis, and the indefinite agreement on the negative AQP4 NMOSD criteria. Collaborative efforts started to take a place among many experts in the region to establish a registry of NMOSD patients for better perception of the disease pattern.

scholarly journals Cohort profile: a collaborative multicentre study of retinal optical coherence tomography in 539 patients with neuromyelitis optica spectrum disorders (CROCTINO)

BMJ Open ◽  
2020 ◽  
Vol 10 (10) ◽  
pp. e035397
Author(s):  
Svenja Specovius ◽  
Hanna G Zimmermann ◽  
Frederike Cosima Oertel ◽  
Claudia Chien ◽  
Charlotte Bereuter ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Neuromyelitis Optica ◽  
Quality Criteria ◽  
Retinal Layer ◽  
Optical Coherence ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Distinct Disease ◽  
History Of ◽  
Spectrum Disorders ◽  
Image Repository

PurposeOptical coherence tomography (OCT) captures retinal damage in neuromyelitis optica spectrum disorders (NMOSD). Previous studies investigating OCT in NMOSD have been limited by the rareness and heterogeneity of the disease. The goal of this study was to establish an image repository platform, which will facilitate neuroimaging studies in NMOSD. Here we summarise the profile of the Collaborative OCT in NMOSD repository as the initial effort in establishing this platform. This repository should prove invaluable for studies using OCT to investigate NMOSD.ParticipantsThe current cohort includes data from 539 patients with NMOSD and 114 healthy controls. These were collected at 22 participating centres from North and South America, Asia and Europe. The dataset consists of demographic details, diagnosis, antibody status, clinical disability, visual function, history of optic neuritis and other NMOSD defining attacks, and OCT source data from three different OCT devices.Findings to dateThe cohort informs similar demographic and clinical characteristics as those of previously published NMOSD cohorts. The image repository platform and centre network continue to be available for future prospective neuroimaging studies in NMOSD. For the conduct of the study, we have refined OCT image quality criteria and developed a cross-device intraretinal segmentation pipeline.Future plansWe are pursuing several scientific projects based on the repository, such as analysing retinal layer thickness measurements, in this cohort in an attempt to identify differences between distinct disease phenotypes, demographics and ethnicities. The dataset will be available for further projects to interested, qualified parties, such as those using specialised image analysis or artificial intelligence applications.

scholarly journals Peripapillary and parafoveal vascular network assessment by optical coherence tomography angiography in aquaporin-4 antibody-positive neuromyelitis optica spectrum disorders

2018 ◽  
Vol 103 (6) ◽  
pp. 789-796 ◽  
Author(s):  
Yongheng Huang ◽  
Lei Zhou ◽  
Jingzi ZhangBao ◽  
Tongjia Cai ◽  
Bei Wang ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Neuromyelitis Optica ◽  
Vessel Density ◽  
Aquaporin 4 ◽  
Optical Coherence ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Spectral Domain Oct ◽  
History Of ◽  
Spectrum Disorders ◽  
Aquaporin 4 Antibody

Background/aimsCurrent understanding of the alterations in the retinal vascular network in neuromyelitis optica spectrum disorders (NMOSDs) is limited. We aim to assess the peripapillary and parafoveal vessel density in aquaporin-4 antibody-positive NMOSD patients by optical coherence tomography (OCT) angiography.MethodsA total of 55 aquaporin-4 antibody-positive NMOSD patients with or without a history of optic neuritis (ON) and 33 healthy controls underwent spectral domain OCT and OCT angiography. Clinical histories, Expanded Disability Status Scale score, visual functional system score (VFSS) and disease duration were collected.ResultsPeripapillary and parafoveal vessel density was significantly decreased in NMOSD eyes with or without a history of ON. The decrease in retinal vessel density could occur before ON and retinal nerve fibre layer (RNFL) atrophy. Peripapillary vessel density correlated well with the spectral domain OCT measurements and VFSS in NMOSD eyes with a history of ON.ConclusionSubclinical primary retinal vasculopathy may occur in NMOSD prior to ON and RNFL atrophy. Peripapillary vessel density might be a sensitive predictor of visual outcomes in NMOSD patients with ON.

Predictors of response to first-line immunosuppressive therapy in neuromyelitis optica spectrum disorders

2017 ◽  
Vol 23 (14) ◽  
pp. 1902-1908 ◽  
Author(s):  
Su-Hyun Kim ◽  
Jae-Won Hyun ◽  
AeRan Joung ◽  
Eun Young Park ◽  
Jungnam Joo ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Poor Response ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
First Line ◽  
Severe Attack ◽  
Treatment History ◽  
Younger Age ◽  
History Of ◽  
Spectrum Disorders ◽  
Pre Treatment

Background: Azathioprine (AZA) and mycophenolate mofetil (MMF) are the most commonly used first-line therapies for patients with neuromyelitis optica spectrum disorders (NMOSD). However, some patients experience a relapse following AZA or MMF treatment. Objectives: To identify factors that predict a response to AZA or MMF in NMOSD. Methods: We retrospectively evaluated medical records from 116 patients who were initially treated with AZA or MMF for at least 6 months. Poor response was defined as ⩾2 relapses or ⩾1 severe relapse. Results: Among the 116 patients, 40 (34%) were classified as poor responders. Logistic regression analyses revealed that a poor response was independently associated with a pre-treatment history of a severe attack ( p < 0.001) and a younger age at disease onset ( p = 0.022). Among the 40 patients with a poor response, 29 (73%) switched to rituximab, and only 3 (10%) had a poor response to rituximab. Conclusion: Patients with a pre-treatment history of a severe attack and a younger age of onset exhibited an increased risk of a poor response to AZA or MMF therapy. Identifying patients who are unlikely to respond to AZA or MMF therapy may allow for treatment with more potent therapies that improve treatment outcomes.

scholarly journals Neuromyelitis optica spectrum disorders and pregnancy: Interactions and management

2017 ◽  
Vol 23 (14) ◽  
pp. 1808-1817 ◽  
Author(s):  
Eslam Shosha ◽  
Sean J Pittock ◽  
Eoin Flanagan ◽  
Brian G Weinshenker
Keyword(s):  
Neuromyelitis Optica ◽  
Treatment Options ◽  
Fetal Loss ◽  
Target Antigen ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Childbearing Age ◽  
Pregnancy And Postpartum ◽  
Changes In Pregnancy ◽  
Spectrum Disorders ◽  
Common Target

Neuromyelitis optica spectrum disorders (NMOSD) predominantly affect women who are of childbearing age. Understanding the interactions between pregnancy and NMOSD is important for clinical management. Aquaporin-4 (AQP4), the most common target antigen in NMOSD, is expressed on placenta in early pregnancy. A variety of immune and cytokine changes in pregnancy may impact pregnancy outcomes in NMOSD patients. Relapses continue during pregnancy and increase in frequency postpartum. Preeclampsia and fetal loss are more frequent in NMOSD than in controls. Transfer of AQP4-immunoglobulin G (IgG) from mother to baby occurs but appears not to cause disease. Several treatment options are relatively safe and mitigate the risk of relapse during pregnancy and postpartum. For patients with active NMOSD, it may be advisable to continue immunotherapy during pregnancy.

P 855. Epidemiology of Neuromyelitis Optica Spectrum Disorders in Children and Adolescents

2018 ◽  
Author(s):  
Christian Lechner ◽  
Matthias Baumann ◽  
Eva-Maria Hennes ◽  
Kathrin Schanda ◽  
Markus Reindl ◽  
...  
Keyword(s):  
Children And Adolescents ◽  
Neuromyelitis Optica ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Spectrum Disorders
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