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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...

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Autores principales: Sokhi, Dilraj, Suleiman, Adil, Manji, Soraiya, Hooker, Juzar, Mativo, Peter
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2021
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.
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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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