Nelson Textbook of Pediatrics 22nd Edition | AQP4 antibody-associated disease (90% of cases) or MOGAD. Core clinical syndromes: optic neuritis, longitudinally extensive transverse myelitis (LETM, ≥3 vertebral segments), area postrema syndrome (intractable nausea/vomiting/hiccups), acute brainstem syndrome, diencephalic syndrome, symptomatic cerebral syndrome. Treatment: acute (IV methylprednisolone, plasma exchange), long-term (rituximab, eculizumab, mycophenolate, azathioprine). High relapse risk.
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