Posts tagged: igg

We reported recently that intracerebral administration of NMO-IgG with human complement produces neuromyelitis optica (NMO) lesions in mice. We examined the role of T cells in the formation of NMO lesions by comparing brain histopathology in wildtype and nude mice. Brains were co-injected with IgG from NMO patients and human complement.

Neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system in which binding of pathogenic autoantibodies (NMO-IgG) to astrocyte aquaporin-4 (AQP4) cause complement-dependent cytotoxicity (CDC) and inflammation.

OBJECTIVE: To investigate the feature brain damage and clinical manifestations in neuromyelitis optica (NMO) patients; To investigate the relationship between serum NMO-IgG antibody and NMO brain damage. METHODS: Clinical data of 37 NMO patients and their head and spinal cord MRI by 1.5T superconducting MR scanner, were analyzed; serum NMO-IgG antibody were measured by immunofluorescence. RESULTS: 17 cases were found to have abnormal signals on MRI, which were mainly in the white matter, pons, medulla, ventricle, aqueduct, and around the corpus callosum; According to pathological changes, brain damage can be divided into scattered irregularity (13 cases), fusion (3 cases), multiple sclerosis-like (1 case), with scattered irregularity more common, 5 cases had clinical manifestations of brain damage: somnolence, vomiting, diplopia, visual rotation, 11 cases patients with brainstem damage show positive serum NMO-IgG antibodies

Aquaporin-4 (AQP4) is a water-selective transporter expressed in astrocytes throughout the central nervous system, as well as in kidney, lung, stomach and skeletal muscle. The two AQP4 isoforms produced by alternative spicing, M1 and M23 AQP4, form heterotetramers that assemble in cell plasma membranes in supramolecular structures called orthogonal arrays of particles (OAPs). Phenotype analysis of AQP4-null mice indicates the involvement of AQP4 in brain and spinal cord water balance, astrocyte migration, neural signal transduction and neuroinflammation.

BACKGROUND: Antibodies targeting membrane proteins play an important role in various autoimmune diseases of the nervous system.

Objectives: the aim of our study was to investigate whether the presence of IgG-NMO antibody is associated with a different pattern of response at visual evoked potentials (VEPs) in patients affected by neuromyelites optica spectrum of disorders (NMOsd) with optic neuritis (ON). Methods: we retrospectively studied clinical, immunological and neurophysiological data from 28 patients affected by NMOsd who presented at least one optic neuritis (16 patients bilateral and 12 unilateral).

Background: Neuromyelitis Optica (NMO) is a demyelinating disease of the central nervous system which preferentially involves the optic nerve and the spinal cord. This is the first inflammatory disease of the CNS in which a specific antibody (NMO-IgG) has been detected.

Background: Longitudinally extensive transverse myelitis (LETM) is defined clinically by acute transverse myelitis (ATM) and radiologically by extensive spinal cord (SC) lesions spanning three or more vertebral segments on Magnetic Ressonance Image (MRI). Even that LETM carries a considerable diagnostic challenge, has been regarded as a spectrum of Neuromyelitis optica (NMO). The seropositivity for NMO IgG, a specific biomarker of NMO, is variable in LETM patients around the world and reach 30-35% in brazilian series.

Background: Neuromyelitis optica (NMO) is characterized by severe optic neuritis and transverse myelitis. A disease-specific autoantibody against aquaporin (AQP) 4, mainly expressed in astrocytic foot processes, was found in the sera from patients with NMO.

In 2004, a highly disease-specific autoantibody named NMO-IgG was discovered in patients with neuromyelitis optica (NMO) and NMO related diseases (i.e. relapsing optic neuritis and longitudinally extensive transverse myelitis). The target antigen of NMO-IgG was identified as aquaporin-4 (AQP4), the main water channel protein in the central nervous system (CNS).

Neuromyelitis optica (NMO or Devic’s syndrome) is a rare autoimmune disease, previously considered a multiple sclerosis variant. The most important laboratory and clinical features are optic myelitis and transverse myelitis, associated with neuromyelitis optica-IgG antibody (NMO-IgG) positivity. Subsequent to this immunological test being available, different groups have described the not-so-rare comorbidity of neuromyelitis optica with other systemic autoimmune diseases, systemic lupus erythematosus with secondary anti-phospholipid syndrome (APS) in particular.

INTRODUCTION: The description of a highly sensitive and specific biomarker for neuromyelitis optica (NMO-IgG/aquaporin-4 antibody) extended the clinical spectrum of NMO to limited forms such as optic neuritis (ON) and longitudinally extensive myelitis (LEM). OBJECTIVE: To asses the sensitivity and specificity of our assay, and to describe the clinical characteristics of the patients who were tested for NMO-IgG. METHODS: NMO-IgG was analysed by immunohistochemistry and confirmed by assay on HEK cells transfected with aquaporin-4.