title: Epidemiological, clinical, and immunological characteristics of neuromyelitis optica: A review.
abstract: The aim of this study was to review the epidemiological and clinical characteristics of neuromyelitis optica (NMO) and the immunopathological mechanisms involved in the neuronal damage. NMO is an inflammatory demyelinating autoimmune disease of the central nervous system that most commonly affects the optic nerves and spinal cord. NMO is thought to be more prevalent among non-Caucasians and where multiple sclerosis (MS) prevalence is low. NMO follows a relapsing course in more than 80-90% of cases, which is more commonly in women. It is a complex disease with an interaction between host genetic and environmental factors and the main immunological feature is the presence of anti-aquaporin 4 (AQP4) antibodies in a subset of patients. NMO is frequently associated with multiple other autoantibodies and there is a strong association between NMO with other systemic autoimmune diseases. AQP4-IgG can cause antibody-dependent cellular cytotoxicity (ADCC) when effector cells are present and complement-dependent cytotoxicity (CDC) when complement is present. Acute therapies, including corticosteroids and plasma exchange, are designed to minimize injury and accelerate recovery. Several aspects of NMO pathogenesis remain unclear. More advances in the understanding of NMO disease mechanisms are needed in order to identify more specific biomarkers to NMO diagnosis.
authors.affiliation: Health Sciences Postgraduate Program, Health Sciences Center, State University of Londrina, Londrina, Paraná 86038-440, Brazil; Outpatient Clinic for Demyelinating Diseases, University Hospital, State University of Londrina, Londrina, Paraná 86061-335, Brazil. Electronic address: wildea_lice@yahoo.com.br.
authors.forename: Wildéa Lice de Carvalho Jennings
authors.initials: WL
authors.lastname: Pereira
authors.affiliation: Department of Pathology, Clinical Analysis and Toxicology, Health Sciences Center, State University of Londrina, Londrina, Paraná 86038-440, Brazil. Electronic address: reiche@sercomtel.com.br.
authors.forename: Edna Maria Vissoci
authors.initials: EM
authors.lastname: Reiche
authors.affiliation: Health Sciences Postgraduate Program, Health Sciences Center, State University of Londrina, Londrina, Paraná 86038-440, Brazil. Electronic address: anapaulakallaur@yahoo.com.br.
authors.forename: Ana Paula
authors.initials: AP
authors.lastname: Kallaur
authors.affiliation: Outpatient Clinic for Demyelinating Diseases, University Hospital, State University of Londrina, Londrina, Paraná 86061-335, Brazil; Department of Clinical Medicine, Health Sciences Center, State University of Londrina, Londrina, Paraná 86038-440, Brazil. Electronic address: rkaimen@uel.br.
authors.forename: Damacio Ramón
authors.initials: DR
authors.lastname: Kaimen-Maciel
mesh_terms: D000906:Antibodies / Q000097:blood; D051401:Aquaporin 4 / Q000276:immunology*; D006801:Humans; D009471:Neuromyelitis Optica / Q000453:epidemiology / Q000276:immunology / Q000628:therapy; D009900:Optic Nerve / Q000473:pathology; D013116:Spinal Cord / Q000473:pathology
chemical_list: D000906:Antibodies; D051401:Aquaporin 4
keywords: Aquaporin 4 antibodies; Autoimmunity; Cytotoxicity; Neuromyelitis optica; Optic nerve; Spinal cord