Autoimmune AQP4 channelopathies and neuromyelitis optica spectrum disorders

SR Hinson, VA Lennon, SJ Pittock - Handbook of clinical neurology, 2016 - Elsevier
Neuromyelitis optica (NMO) spectrum disorders (SD) represent an evolving group of central
nervous system (CNS)-inflammatory autoimmune demyelinating diseases unified by a
pathogenic autoantibody specific for the aquaporin-4 (AQP4) water channel. It was
historically misdiagnosed as multiple sclerosis (MS), which lacks a distinguishing biomarker.
The discovery of AQP4-IgG moved the focus of CNS demyelinating disease research from
emphasis on the oligodendrocyte and myelin to the astrocyte. NMO is recognized today as a …