Radiologically isolated aquaporin-4 antibody neuromyelitis optica spectrum disorder

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.

Automated summary

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.