Pathogenic antibodies to AQP4: Neuromyelitis optica spectrum disorder (NMOSD)

• Published in: Biochimica et biophysica acta. Biomembranes Vol. 1863; no. 12; p. 183772
• Main Authors: Wright, Sukhvir K., Wassmer, Evangeline, Vincent, Angela
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.12.2021
• Subjects: Antibodies - genetics; Antibodies - immunology; AQP4; Aquaporin 4 - genetics; Aquaporin 4 - immunology; Central Nervous System - immunology; Central Nervous System - pathology; Humans; Immunoglobulin G - genetics; Immunoglobulin G - immunology; Immunotherapy; MOGAD; Neuroinflammation; Neuromyelitis Optica - genetics; Neuromyelitis Optica - immunology; Neuromyelitis Optica - pathology; Neuronal antibodies; NMOSD; Optic Nerve - immunology; Optic Nerve - pathology; Spinal Cord - immunology; Spinal Cord - pathology; Neuronal antibodies; LETM; Immunotherapy; CSF; CNS; Neuroinflammation; AQP4; MOGAD; NMOSD
• ISSN: 0005-2736; 1879-2642
• DOI: 10.1016/j.bbamem.2021.183772

NMOSD is a rare but severe relapsing remitting demyelinating disease that affects both adults and children. Most patients have pathogenic antibodies that target the central nervous system AQP4 protein. This review provides an update on our current understanding of the disease pathophysiology and describes the clinical, paraclinical features and therapeutic management of the disease. [Display omitted] •Antibodies to Aquaporin-4 are found in Neuromyelitis spectrum disorder (NMOSD).•Patients suffer from recurrent attacks of neuroinflammation affecting the spinal cord and optic nerves.•There is no cure for NMOSD at present; treatment is aimed at treating acute attacks and preventing future relapses.