• Autoimmune glial fibrillary acidic protein astrocytopathy is a form of monophasic or relapsing steroid-responsive meningoencephalomyelitis often accompanied by a hallmark radial periventricular brain MRI enhancement pattern and inflammatory cerebrospinal fluid with lymphocytic pleocytosis. (medlink.com)
  • Autoimmune glial fibrillary acidic protein astrocytopathy (A-GFAP-A) is a newly discovered autoimmune nervous system disease involving the brain, spinal cord, meninges and optic nerve. (biomedcentral.com)
  • Neuromyelitis optica spectrum disorders (NMOSD), including neuromyelitis optica (NMO), are autoimmune diseases characterized by acute inflammation of the optic nerve (optic neuritis, ON) and the spinal cord (myelitis). (wikipedia.org)
  • BACKGROUND: The effect of smoking on the resolution of magnetic resonance imaging (MRI) lesions in patients with neuromyelitis optica spectrum disorders with aquaporin-4 positive antibody (NMOSD-AQP4) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) has not been studied before. (bvsalud.org)
  • BACKGROUND: Patients with anti-aquaporin-4 antibody seropositive (AQP4-IgG+) neuromyelitis optica spectrum disorders (NMOSDs) frequently suffer from optic neuritis (ON) leading to severe retinal neuroaxonal damage. (ox.ac.uk)
  • Autoimmune GFAP astrocytopathy manifests as a meningoencephalomyelitis (or limited form thereof) with dramatic steroid-responsiveness. (medlink.com)
  • Autoimmune GFAP astrocytopathy presentation is subacute (over days to a few weeks). (medlink.com)
  • Lesions may also affect the diencephalon, mostly in Aquaporin-4-Immunoglobulin-G (AQP4-IgG) NMOSD. (wikipedia.org)
  • NMOSD is caused by an autoimmune attack on the nervous system. (wikipedia.org)
  • citation needed] NMOSD is usually caused by autoantibodies targeting aquaporin 4 (AQP4), a channel protein in the cell membrane that allows water to pass through the membrane. (wikipedia.org)
  • Thus, NMOSD involving AQP4-IgG can be considered an astrocytopathy or autoimmune astrocytic channelopathy, since the astrocytes are semi-selectively destroyed. (wikipedia.org)
  • 2 3 4 5 6 ] However, a more recent population-based survey conducted in urban Mangalore has shown a prevalence of 8.3/100,000 for MS and 2.6/100,000 for neuromyelitis optic spectrum diseases (NMOSD). (lww.com)
  • The overlapping clinical features of relapsing remitting multiple sclerosis (RRMS), aquaporin-4 (AQP4)-antibody (Ab) neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein (MOG)-Ab disease mean that detection of disease specific serum antibodies is the gold standard in diagnostics. (biomedcentral.com)
  • The anti-mog spectrum in children is equally variated: Out of a sample of 41 children with MOG-antibodies 29 had clinical NMOSD (17 relapsing), 8 had ADEM (4 relapsing with ADEM-ON), 3 had a single clinical event CIS, and 1 had a relapsing tumefactive disorder. (mdwiki.org)
  • Further, the relationship of this retinal damage to a primary astrocytopathy in NMOSD is uncertain. (ox.ac.uk)
  • In a study published this month in Annals of Neurology , researchers from Osaka University have revealed that treatment with an antibody to a protein called repulsive guidance molecule-a (RGMa) dramatically improves symptoms of neuromyelitis optica , a devastating autoimmune disorder, in an experimental rat model. (medicalxpress.com)
  • Shosuke Iwamoto et al, RGMa Signal in Macrophages Induces Neutrophil‐Related Astrocytopathy in NMO, Annals of Neurology (2022). (medicalxpress.com)
  • Anti-MOG associated spectrum , often clinically presented as an anti- MOG autoimmune encephalomyelitis , [21] [22] but can also appear as negative NMO or atypical multiple sclerosis. (mdwiki.org)
  • They are sometimes considered different diseases from Multiple Sclerosis, [4] [5] but considered by others to form a spectrum differing only in terms of chronicity, severity, and clinical course. (mdwiki.org)
  • In more than 80% of cases, IgG autoantibodies against aquaporin-4 (anti-AQP4+) are the cause, and in 10-40% of the remaining cases, IgG antibodies against MOG are the cause. (wikipedia.org)
  • In most cases, NMO is caused by antibodies that the body develops to a protein called aquaporin-4 (AQP4). (medicalxpress.com)
  • For the primary analysis, patients will subsequently be assigned into the MS-ON group, the aquapotin-4-IgG positive ON (AQP4-IgG+ON) group or the MOG-IgG positive ON (MOG-IgG+ON) group and then further sub-stratified according to the number of days from the onset of visual loss to high-dose corticosteroids (days-to-Rx). (bvsalud.org)
  • Rarely, NMO may occur in the context of other autoimmune diseases (e.g. connective tissue disorders, paraneoplastic syndromes) or infectious diseases. (wikipedia.org)
  • Longitudinally extensive myelitis or optic neuritis associated with systemic autoimmune disease. (mdwiki.org)
  • Autoimmune diseases are the molecular equivalent of "friendly fire": The body attacks itself instead of harmful invaders. (medicalxpress.com)
  • In more than 80% of cases, NMO is caused by immunoglobulin G autoantibodies to aquaporin 4 (anti-AQP4), the most abundant water channel protein in the central nervous system. (wikipedia.org)
  • Accumulation of these mediators culminates in chronic neuroinflammation that exacerbates AD pathogenesis [ 4 , 10 ]. (biomedcentral.com)
  • In more than 80% of cases, NMO is caused by immunoglobulin G autoantibodies to aquaporin 4 (anti-AQP4), the most abundant water channel protein in the central nervous system. (wikipedia.org)
  • Lesions may also affect the diencephalon, mostly in Aquaporin-4-Immunoglobulin-G (AQP4-IgG) NMOSD. (wikipedia.org)
  • citation needed] NMOSD is usually caused by autoantibodies targeting aquaporin 4 (AQP4), a channel protein in the cell membrane that allows water to pass through the membrane. (wikipedia.org)
  • Thus, NMOSD involving AQP4-IgG can be considered an astrocytopathy or autoimmune astrocytic channelopathy, since the astrocytes are semi-selectively destroyed. (wikipedia.org)
  • Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy against foot processes of aquaporin-4 (AQP4) water channels. (nih.gov)
  • The atrophy rate in patients with anti-aquaporin-4 antibody-positive NMOSD (AQP4 + NMOSD) was compared with age-sex-matched HCs recruited from the Japanese Alzheimer's Disease Neuroimaging Initiative study and another study performed at Chiba University. (bvsalud.org)
  • Background: Stroke stimulates reactive astrogliosis, aquaporin 4 (AQP4) depolarization and neuroinflammation. (bvsalud.org)
  • Neuromyelitis optica spectrum disorders (NMOSD) are severe inflammatory disorders of the central nervous system targeting aquaporin‐4 (AQP4). (nature.com)
  • Satralizumab-mwge, marketed as Enspryng, was approved for adults with NMOSD who have antibodies against the aquaporin 4 (AQP4) protein. (autoimmune-encephalitis.org)
  • Neuromyelitis optica spectrum disorders (NMOSD), including neuromyelitis optica (NMO), are autoimmune diseases characterized by acute inflammation of the optic nerve (optic neuritis, ON) and the spinal cord (myelitis). (wikipedia.org)
  • Neuromyelitis optica (NMO) is a chronic, autoimmune inflammatory, astrocyte depleting and demyelinating disease of the central nervous system (CNS) and typically affects the spinal cord (myelitis) and optic nerves (neuritis nervi optici). (uni-goettingen.de)
  • Recently they have also contributed to the discovery of the third autoantigen in MG, the LRΡ4, and have discovered a new autoantigen for neuromyelitis optica, the aquaporin-1 . (neurodiagnostics.gr)
  • 2014), and of the anti-aquaporin-1 antibodies in neuromyelitis optica (J. Tzartos et al. (neurodiagnostics.gr)
  • In more than 80% of cases, IgG autoantibodies against aquaporin-4 (anti-AQP4+) are the cause, and in 10-40% of the remaining cases, IgG antibodies against MOG are the cause. (wikipedia.org)
  • Rarely, NMO may occur in the context of other autoimmune diseases (e.g. connective tissue disorders, paraneoplastic syndromes) or infectious diseases. (wikipedia.org)
  • The pathogenesis of NMO is based on autoimmune astrocytopathy, due to IgG-Ak against AQP-4 water channels localized at the perivascular astrocyte foot processes. (uni-goettingen.de)
  • Astrocyte-selective ablation of Hmgb1 at birth affects astrocyte morphology and endfoot placement, alters distribution of endfoot proteins connexin43 and aquaporin-4, induces transcriptional changes in astrocytes related to cytoskeleton remodeling, and profoundly disrupts endothelial ultrastructure. (bvsalud.org)
  • To report the frequency of area postrema syndrome (APS) in glial fibrillary acidic protein-immunoglobulin G (GFAP-IgG)-positive patients and emphasize the importance of APS among the phenotypes in autoimmune GFAP astrocytopathy. (nih.gov)
  • In more than two thirds of patients, IgG against the water channel aquaporin-4 (AQP-4) can be detected in the serum. (uni-goettingen.de)
  • They further observed loss of WISW in 3 diabetic patients and evidence of autonomic failure but not peripheral neuropathy and in a patient with Guillain-Barre polyneuropathy [4]. (irispublishers.com)
  • Since the publication of the original McDonald criteria[3] and subsequent iterations,[4, 5, 6] radiologic endpoints play an important role in the diagnosis of MS. (medscape.com)