Clinical Usefulness of Cell-based Indirect Immunofluorescence Assay for the Detection of Aquaporin-4 Antibodies in Neuromyelitis Optica Spectrum Disorder Eun-suk Kang, M.D.,1 Ju-Hong Min, M.D.,2 Kwang Ho Lee, M.D.,2 and Byoung Joon Kim, M.D.2 Author information ? Article notes ? Copyright and License information ? Go to: Abstract. Background The presence of antibodies to aquaporin-4 (AQP4) has been identified as a key characteristic of neuromyelitis optica spectrum disorder (NMOSD), an autoimmune inflammatory demyelinating central nervous system (CNS) disorder. We evaluated the performance of a cell-based indirect immunofluorescence assay (CIIFA) for detecting AQP4 antibodies using antigen prepared with a recombinant AQP4 peptide transfection technique and assessed the usefulness of CIIFA for diagnosis of NMOSD in routine clinical practice.
Abstract OBJECTIVE: Neuromyelitis optica spectrum disorder (NMOsd) is an inflammatory and demyelinating syndrome characterized by optic neuritis and myelitis. Several magnetization transfer MRI studies have revealed abnormalities in normal-appearing gray matter in NMOsd. The aim of this study is to elucidate the characteristics and pathogenesis of cognitive impairment and neurodegeneration in NMOsd brains
Aquaporin 4-specific T cells in neuromyelitis optica exhibit a Th17 bias and recognize Clostridium ABC transporter. Varrin-Doyer M, Spencer CM, Schulze-Topphoff U, Nelson PA, Stroud RM, C Cree BA, Zamvil SS.
Clinical Study Brain MRI abnormalities in Brazilian patients with neuromyelitis optica Cíntia Elias Piresa, b, , , Christianne Martins Correa da Silvaa, Fernanda Cristina Rueda Lopesa, b, Fabiola Rachid Malfetanoa, Valéria C.S.R. Pereiraa, Tadeu Kubob, Paulo R.V. Bahiaa, Soniza Vieira Alves-Leona, Emerson L.
Genetic analysis of aquaporin-4 in neuromyelitis optica. Matiello M , Schaefer-Klein JL , Hebrink DD , Kingsbury DJ , Atkinson EJ , Weinshenker BG ; On behalf of the NMO Genetics Collaborators
Fazio R et al. – Neuromyelitis optica (NMO) is a rare demyelinating disease, affecting selectively the optic nerve and the spinal cord. It was previously considered to be a severe variant of multiple sclerosis (MS) due to the similar pathological features and its resemblance to optico–spinal, or Japanese, MS, typical of Asian populations.
Devic’s syndrome was first described in 1870 by Sir Thomas Allbutt (what a name to have had as a kid!) who pointed out an association between myelitis and optic nerve disorder.? In 1894, Eugene Devic and his student Fernand Gault described 16 patients who had lost vision in one or both eyes and developed spastic weakness, sensory loss, and incontinence.? The other name of the condition was neuromyelitis optica (NMO). A major breakthrough came in 2004 when a specific marker NMO-IgG was found for the disorder [1].? IgG stands for immunoglobulin (a kind of antibody).
SPECIAL SECTION: MONOCLONAL ANTIBODIES WHAT ARE MONOCLONAL ANTIBODIES (MABs)?
BACKGROUND: Antibodies to the water channel protein aquaporin-4 (AQP4), which is expressed in astrocytic endfeet at the blood brain barrier, have been identified in the serum of Neuromyelitis optica (NMO) patients and are believed to induce damage to astrocytes. However, AQP4 specific T helper cell responses that are required for the generation of anti-AQP4 antibodies and most likely also for the formation of intraparenchymal CNS lesions have not been characterized. METHODOLOGY/PRINCIPAL FINDINGS: Using overlapping 15-meric peptides of AQP4, we identified the immunogenic T cell epitopes of AQP4 that are restricted to murine major histocompatibility complex (MHC) I-A(b).
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).
Objective: Clinical studies indicate that anti-CD20 B-cell depletion may be an effective multiple sclerosis (MS) therapy. We investigated mechanisms of anti-CD20-mediated immune modulation using 2 paradigms of experimental autoimmune encephalomyelitis (EAE). Methods: Murine EAE was induced by recombinant myelin oligodendrocyte glycoprotein (rMOG), a model in which B cells are considered to contribute pathogenically, or MOG peptide (p)35-55, which does not require B cells.
Astrocytes are specialized glial cells that outnumber neurons by over fivefold. They contiguously tile the entire central nervous system (CNS) and exert many essential complex functions in the healthy CNS