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Neuromyelitis Optica Spectrum Disorder — SCE Neurology MCQ

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HardMultiple Sclerosis & CNS InflammationNeuromyelitis Optica Spectrum DisorderSCE Neurology

A patient has sequential severe optic neuritis and longitudinally extensive transverse myelitis. Brain MRI is not typical for multiple sclerosis, CSF lacks unmatched oligoclonal bands and aquaporin-4 IgG is positive. What is the diagnosis?

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Correct answer: C — Aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder

The best answer is “Aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder”. The AQP4 antibody and core opticospinal syndromes establish NMOSD and have important acute and preventive treatment implications. “Relapsing-remitting multiple sclerosis” is a credible alternative elsewhere, but it fails the decisive discriminator in this case. “MOG-antibody-associated disease” is a credible alternative elsewhere, but it fails the decisive discriminator in this case. “Acute disseminated encephalomyelitis” is a credible alternative elsewhere, but it fails the decisive discriminator in this case. “Primary CNS lymphoma” is a credible alternative elsewhere, but it fails the decisive discriminator in this case.

Reference: International consensus diagnostic criteria for NMOSD: https://pubmed.ncbi.nlm.nih.gov/26092914/