title: GAD65 neurological autoimmunity.
abstract: The glutamic acid decarboxylase 65-kilodalton isoform (GAD65) antibody is a biomarker of autoimmune central nervous system (CNS) disorders and, more commonly, nonneurological autoimmune diseases. Type 1 diabetes, autoimmune thyroid disease, and pernicious anemia are the most frequent GAD65 autoimmune associations. One or more of these disorders coexists in approximately 70% of patients with GAD65 neurological autoimmunity. Neurological phenotypes have CNS localization and include limbic encephalitis, epilepsy, cerebellar ataxia, and stiff-person syndrome (SPS), among others. Classic SPS is a disorder on the spectrum of CNS hyperexcitability which also includes phenotypes that are either more restricted (stiff-limb syndrome) or more widespread (progressive encephalomyelitis with rigidity and myoclonus). GAD65 antibody is not highly predictive of a paraneoplastic cause for neurological disorders, but diverse cancer types have been occasionally reported. For all phenotypes, responses to immunotherapy are variable (approximately 50% improve). GAD65 autoimmunity is important to recognize for both coexisting nonneurological autoimmune associations and potential immunotherapy-response. Muscle Nerve 56: 15-27, 2017.
authors.affiliation: Department of Neurology, College of Medicine, Mayo Clinic, 200 1st Street SW, Rochester, Minnesota, 55905, USA.|Department of Laboratory Medicine and Pathology, College of Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Andrew
authors.initials: A
authors.lastname: McKeon
authors.affiliation: Department of Neurology, College of Medicine, Mayo Clinic, 200 1st Street SW, Rochester, Minnesota, 55905, USA.
authors.forename: Jennifer A
authors.initials: JA
authors.lastname: Tracy
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000276:immunology / Q000378:metabolism*; D020278:Demyelinating Autoimmune Diseases, CNS / Q000276:immunology* / Q000378:metabolism*; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans
chemical_list: D001323:Autoantibodies; D005968:Glutamate Decarboxylase; C401141:glutamate decarboxylase 2
keywords: GAD65; ataxia; autoimmune; neurological; stiff-person