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Seropositive neuromyelitis optica following herpes zoster and SARS-COV-2 infections

Bakheet, Mashair
Laws, Elinor
Martinez-Alvarez, Lidia
Blanch, Richard J
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University Hospitals Birmingham NHS Foundation Trust ;Royal Centre for Defence Medicine; Sandwell and West Birmingham NHS Trust
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2022-06
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Abstract
Background and Aims: Neuromyelitis optica spectrum disorder (NMOSD) is uncommon autoimmune central nervous system disease, with classic presentations of myelitis, optic neuritis, and/or area postrema syndrome. Approximately 75% of patients have aquaporin 4 (AQP4) antibodies. It is treated with gluco-corticoids and often plasma exchange; and long-term immunosuppression is recommended. Different factors are involved in pathogenesis including infectionsMethods: In this case report, a patient who had suffered from Herpes zoster (HZV) and SARS-COV-2 infections, developed later NMOSD which has been confirmed with the clinical and radiological findings along with positive serum AQP4.Results: A 55-year-old African woman presented with an upper thoracic HZV rash and facial paresis, which quickly progressed into an area postrema syndrome, followed by encephalitis, bilateral visual loss and quadriplegia. Earlier presenting signs were masked by SARS-COV-2 and concomitant HZV infections. Initial magnetic resonance imaging (MRI) of her brain was inconclusive. However, clinical progression and a repeat head and spine MRI with contrast revealed features highly suggestive of NMOSD, supported with positive AQP4 serology. Despite aggressive treatment with high dose glucocorticoids, plasma exchange, intravenous immunoglobulin and cyclophosphamide, she retained profound neurological deficits with partial improvement only.Conclusions: This is a unique case where both HZV and SARS-COV-2 infections immediately preceded a devastating NMOSD presentation. This case highlights the importance of early and aggressive treatment in NMOSD with additional diagnostic pitfalls when co-existent with an infectious trigger.Disclosures: None
Citation
Mashair Bakheet, Elinor Laws, Lidia Martinez-Alvarez, Richard J Blanch. Seropositive neuromyelitis optica following herpes zoster and SARS-COV-2 infections. Neuro-Neuro-Ophthalmology. 2022, Jun (46):1-139. https://doi.org/10.1080/01658107.2022.2064188
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