title: Immunotherapy in Peripheral Neuropathies.
abstract: Immunotherapy has been investigated in a small subset of peripheral neuropathies, including an acute one, Guillain-Barré syndrome, and 3 chronic forms: chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy, and neuropathy associated with IgM anti-myelin-associated glycoprotein. Several experimental studies and clinical data are strongly suggestive of an immune-mediated pathogenesis. Either cell-mediated mechanisms or antibody responses to Schwann cell, compact myelin, or nodal antigens are considered to act together in an aberrant immune response to cause damage to peripheral nerves. Immunomodulatory treatments used in these neuropathies aim to act at various steps of this pathogenic process. However, there are many phenotypic variants and, consequently, there is a significant difference in the response to immunotherapy between these neuropathies, as well as a need to improve our knowledge and long-term management of chronic forms.
authors.affiliation: National Referral Center for Rare Neuromuscular Diseases, Institut Hospitalo-Universitaire de Neurosciences, University Hospital Pitié-Salpêtrière and University Pierre et Marie Curie (Paris VI), Paris, France. jean-marc.leger@aphp.fr.
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Léger
authors.affiliation: National Referral Center for Rare Neuromuscular Diseases, Institut Hospitalo-Universitaire de Neurosciences, University Hospital Pitié-Salpêtrière and University Pierre et Marie Curie (Paris VI), Paris, France.
authors.forename: Raquel
authors.initials: R
authors.lastname: Guimarães-Costa
authors.affiliation: National Referral Center for Rare Neuromuscular Diseases, Institut Hospitalo-Universitaire de Neurosciences, University Hospital Pitié-Salpêtrière and University Pierre et Marie Curie (Paris VI), Paris, France.
authors.forename: Cristina
authors.initials: C
authors.lastname: Muntean
mesh_terms: D020275:Guillain-Barre Syndrome / Q000628:therapy; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007167:Immunotherapy; D010523:Peripheral Nervous System Diseases / Q000628:therapy*; D010951:Plasma Exchange; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000628:therapy
chemical_list: D007155:Immunologic Factors
keywords: Chronic inflammatory demyelinating polyradiculoneuropathy; Guillain-Barré syndrome; IgM anti-myelin-associated-glycoprotein neuropathy; Immune-mediated neuropathies; Immunomodulatory treatments; Multifocal motor neuropathy