Ever since the discovery of pathogenic aquaporin 4-specific antibodies in the serum of patients with neuromyelitis optica spectrum disorders current knowledge about clinical observations and diagnosis, and about the underlying pathology and resulting therapies have been put forward in excellent reviews and primary publications. However, in order to further develop novel strategies for the treatment of this disease, there is an urgent need to understand the immunological processes associated with the formation of the pathogenic antibodies, and with aberrant immune responses observed in affected patients. In this review, we will highlight and evaluate important studies on these processes.

Neuromyelitis optica spectrum disorders

Aquaporin 4

Spinal cord

Optic nerves

Medulla

Astrocytes

Antibodies

Lymphocytes

Macrophages

Microglia

Complement

Therapies

© 2025 The Author(s). Published by Elsevier Ltd.