Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\26185772
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Neurol+Neuroimmunol+Neuroinflamm
2015 ; 2
(4
): e110
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Aquaporin-4 autoimmunity
#MMPMID26185772
Zekeridou A
; Lennon VA
Neurol Neuroimmunol Neuroinflamm
2015[Aug]; 2
(4
): e110
PMID26185772
show ga
Neuromyelitis optica (NMO) and a related spectrum of inflammatory CNS disorders
are unified by detection of a serum autoantibody specific for the aquaporin-4
(AQP4) water channel, which is abundant in astrocytic foot processes. The classic
clinical manifestations of NMO are optic neuritis and longitudinally extensive
transverse myelitis. Newly recognized manifestations of AQP4 autoimmunity include
lesions of circumventricular organs and skeletal muscle. NMO is commonly
relapsing, is frequently accompanied by other autoimmune disorders, and sometimes
occurs in a paraneoplastic context. The goals of treatment are to minimize
neurologic disability in the acute attack and thereafter to prevent relapses and
cumulative disability. The disease specificity of AQP4 immunoglobulin (Ig) G
approaches 100% using optimized molecular-based detection assays. Clinical,
immunohistopathologic, and in vitro evidence support this antibody being central
to NMO pathogenesis. Current animal models yield limited histopathologic
characteristics of NMO, with no clinical deficits to date. Recent descriptions of
a myelin oligodendrocyte glycoprotein autoantibody in a minority of patients with
NMO spectrum phenotype who lack AQP4-IgG predict serologic delineation of
additional distinctive disease entities.