Journal
MULTIPLE SCLEROSIS JOURNAL
Volume 28, Issue 4, Pages 676-679Publisher
SAGE PUBLICATIONS LTD
DOI: 10.1177/13524585221074947
Keywords
Neuromyelitis optica spectrum disorder; aquaporin-4 antibody; paediatric demyelination; radiologically isolated syndrome
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This study presents a case of radiologically isolated longitudinally extensive optic neuritis in a 12-year-old female with positive serum AQP4-Ab. The patient was asymptomatic and showed resolution of imaging changes after immune therapy. The discussion highlights the pathogenicity of AQP4-Ab in sub-clinical disease and the need for prognostic biomarkers to guide optimal therapy for patients with AQP4-Ab NMOSD.
Aquaporin-4 antibody (AQP4-Ab) Neuromyelitis Optica Spectrum Disorder (NMOSD) is a rare neuroinflammatory syndrome presenting predominantly with optic neuritis and transverse myelitis. We report a case of radiologically isolated longitudinally extensive optic neuritis in an asymptomatic 12-year-old female with positive serum AQP4-Ab, with resolution of imaging changes after immune therapy. By contrast to patients with radiologically isolated syndrome, of which some will never convert to multiple sclerosis, the pathogenicity of AQP4-Ab in the context of sub-clinical disease, supported treatment in our patient. Given the severe morbidity in AQP4-Ab NMOSD, prognostic biomarkers for disease severity are required to guide optimal therapy for patients.
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