NEUROMYELITIS OPTICA: AN EVOLVING SPECTRUM OF DISEASE
Satoshi Kuwabara (Japan)

Neuromyelitis optica spectrum disorders (NMOSD) are relapsing inflammatory diseases. Serum antibodies against the aquaporin-4 (AQP-4) water channel cause recurrent attacks of optic neuritis, myelitis and/or brainstem characteristic syndromes. Associations between optic nerve and spinal cord disease were noted in the 19th century, especially after Devic’s description of acute neuromyelitis optica.

Previous studies of AQP4-IgG–seropositive patients have also shown many additional clinical and neuroimaging findings are present, including brain involvement, which are captured by the term NMOSD. The AQP4 antibodies are positive in approximately 80% of patients with NMOSD and are pathogenic for the inflammatory CNS lesions and clinical manifestations of the disorder.

More recently, there is a subgroup of patients with NMOSD symptoms who are positive for antibodies against myelin-oligodendrocyte-glycoprotein, and the disorder is currently termed MOG antibody-associated disorder (MOGAD).

This lecture will present an overview on current status concerning pathophysiology, diagnostic biomarkers, neuroimaging (typical and atypical), and treatment of NMOSD and MOGAD.