Since the discovery in 2004 of NMO-IgG, the autoantibody associated with neuromyelitis optica (NMO),1 neurologists are increasingly relying on the NMO-IgG test to rule in or rule out NMO. Related disorders like optic neuritis (ON), transverse myelitis (TM), acute disseminated encephalomyelitis (ADEM), and longitudinally extensive TM (LETM; 3 spinal cord vertebral segments) can all be monophasic or multiphasic, can occur together or individually, and can occur in adults or children. Similarly, multiple sclerosis (MS) is often confused with these disorders, especially early in the disease.
Anti-aquaporin-4 antibody (NMO-IgG) is used as a diagnostic marker for neuromyelitis optica (NMO). Although the mechanism of spinal cord lesions in NMO has been investigated, that of extensive hemispheric lesions with brain edema remains unclear.
Discovery of aquaporin water channel proteins has provided insight into the molecular mechanism of membrane water permeability. In mammalian brain, Aquaporin-4 (AQP4) is the main water channel and is distributed with highest density in the perivascular and subpial astrocyte end-feet. AQP4 is a critical component of an integrated water and potassium homeostasis.
OBJECTIVE:To investigate the characteristics of the linear lesions and longitudinally extensive spinal cord (LESC) lesions in Chinese patients with neuromyelitis optica (NMO).
Neuromyelitis optica (NMO), characterised by longitudinally extensive transverse myelitis (LETM), was previously thought to be a variant of multiple sclerosis.
BACKGROUND: Neuromyelitis optica (NMO) is a serious idiopathic inflammatory demyelinating disorder characterized by acute transverse myelitis and optic neuritis. A significant proportion of NMO patients are seropositive for NMO-IgG, an autoantibody targeting aquaporin-4 (AQP4) water channel. Paraneoplastic NMO associated various tumors were recently reported.
BACKGROUND: In 70-80% of cases, neuromyelitis optica (NMO) is associated with highly specific serum auto-antibodies to aquaporin-4 (termed AQP4-Ab or NMO-IgG).
Devic neuromyelitis optica (DNMO) is a severe demyelinating disease of the central nervous system (CNS).