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Cases of neuromyelitis optica spectrum disorder from the East Africa region, highlighting challenges in diagnostics and healthcare access
BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is an auto-immune disease of the central nervous system (CNS) associated with the IgG-antibody against aquaporin-4 (AQP4-IgG). There is little published epidemiology of NMOSD from sub-Saharan Africa (SSA). METHODS: We retrospectively collate...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7844578/ https://www.ncbi.nlm.nih.gov/pubmed/33553703 http://dx.doi.org/10.1016/j.ensci.2021.100320 |
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author | Sokhi, Dilraj Suleiman, Adil Manji, Soraiya Hooker, Juzar Mativo, Peter |
author_facet | Sokhi, Dilraj Suleiman, Adil Manji, Soraiya Hooker, Juzar Mativo, Peter |
author_sort | Sokhi, Dilraj |
collection | PubMed |
description | BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is an auto-immune disease of the central nervous system (CNS) associated with the IgG-antibody against aquaporin-4 (AQP4-IgG). There is little published epidemiology of NMOSD from sub-Saharan Africa (SSA). METHODS: We retrospectively collated NMOSD cases admitted to our tertiary regional neurology centre. RESULTS: We identified 11 cases (10 female, average age 30 years). 64% (7/11) were seropositive for AQP4-IgG, measured using indirect immunofluorescence. The remaining cases could either not afford tests, or had pathognomonic radiological features. 57% (4/7) of seropositive cases had concurrent/recent CNS infection. All patients were treated with high-dose intravenous methylprednisolone (IVMP), and 36% (4/11) also had plasma exchange. Only 55% (6/11) of the patients were seen by a neurologist at presentation: they had less relapses (1.3 vs 2.4), less diagnostic delay (2.3 vs 7.4 months), and were less disabled at the end of our review period. 10 cases were immunosuppressed long-term: 60% on mycophenolate, 30% azathioprine, and one on rituximab. CONCLUSION: Our study is the largest case series of NMOSD from the East Africa region. Patients faced challenges of access to appropriate and affordable testing, and timely availability of a neurologist at onset, which had impacts on their functional outcomes. The majority of the seropositive cases had recent/concurrent CNS infections, suggesting triggered auto-immunity. |
format | Online Article Text |
id | pubmed-7844578 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-78445782021-02-04 Cases of neuromyelitis optica spectrum disorder from the East Africa region, highlighting challenges in diagnostics and healthcare access Sokhi, Dilraj Suleiman, Adil Manji, Soraiya Hooker, Juzar Mativo, Peter eNeurologicalSci Original Article BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is an auto-immune disease of the central nervous system (CNS) associated with the IgG-antibody against aquaporin-4 (AQP4-IgG). There is little published epidemiology of NMOSD from sub-Saharan Africa (SSA). METHODS: We retrospectively collated NMOSD cases admitted to our tertiary regional neurology centre. RESULTS: We identified 11 cases (10 female, average age 30 years). 64% (7/11) were seropositive for AQP4-IgG, measured using indirect immunofluorescence. The remaining cases could either not afford tests, or had pathognomonic radiological features. 57% (4/7) of seropositive cases had concurrent/recent CNS infection. All patients were treated with high-dose intravenous methylprednisolone (IVMP), and 36% (4/11) also had plasma exchange. Only 55% (6/11) of the patients were seen by a neurologist at presentation: they had less relapses (1.3 vs 2.4), less diagnostic delay (2.3 vs 7.4 months), and were less disabled at the end of our review period. 10 cases were immunosuppressed long-term: 60% on mycophenolate, 30% azathioprine, and one on rituximab. CONCLUSION: Our study is the largest case series of NMOSD from the East Africa region. Patients faced challenges of access to appropriate and affordable testing, and timely availability of a neurologist at onset, which had impacts on their functional outcomes. The majority of the seropositive cases had recent/concurrent CNS infections, suggesting triggered auto-immunity. Elsevier 2021-01-23 /pmc/articles/PMC7844578/ /pubmed/33553703 http://dx.doi.org/10.1016/j.ensci.2021.100320 Text en © 2021 The Author(s) http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Original Article Sokhi, Dilraj Suleiman, Adil Manji, Soraiya Hooker, Juzar Mativo, Peter Cases of neuromyelitis optica spectrum disorder from the East Africa region, highlighting challenges in diagnostics and healthcare access |
title | Cases of neuromyelitis optica spectrum disorder from the East Africa region, highlighting challenges in diagnostics and healthcare access |
title_full | Cases of neuromyelitis optica spectrum disorder from the East Africa region, highlighting challenges in diagnostics and healthcare access |
title_fullStr | Cases of neuromyelitis optica spectrum disorder from the East Africa region, highlighting challenges in diagnostics and healthcare access |
title_full_unstemmed | Cases of neuromyelitis optica spectrum disorder from the East Africa region, highlighting challenges in diagnostics and healthcare access |
title_short | Cases of neuromyelitis optica spectrum disorder from the East Africa region, highlighting challenges in diagnostics and healthcare access |
title_sort | cases of neuromyelitis optica spectrum disorder from the east africa region, highlighting challenges in diagnostics and healthcare access |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7844578/ https://www.ncbi.nlm.nih.gov/pubmed/33553703 http://dx.doi.org/10.1016/j.ensci.2021.100320 |
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