title: Clinico-pathological correlation in adenylate kinase 5 autoimmune limbic encephalitis.
abstract: Autoantibodies associated with autoimmune limbic encephalitis (ALE) have been well-characterized, with intracellular neuronal antibodies being less responsive to immunotherapy than antibodies to cell surface antigens. Adenylate kinase 5 (AK5) is a nucleoside monophosphate kinase vital for neuronal-specific metabolism and is located intracellularly in the cytosol and expressed exclusively in the brain. Antibodies to AK5 had been previously identified but were not known to be associated with human disease prior to the report of two patients with AK5-related ALE (Tuzun et al., 2007). We present the complete clinical picture for one of these patients and the first reported neuropathology for AK5 ALE.
authors.affiliation: Memory and Aging Center, University of California, San Francisco, Sandler Neurosciences Centre, 675 Nelson Rising Lane, Suite 190, San Francisco, CA 94158, USA.
authors.forename: Adeline S L
authors.initials: AS
authors.lastname: Ng
authors.affiliation: Memory and Aging Center, University of California, San Francisco, Sandler Neurosciences Centre, 675 Nelson Rising Lane, Suite 190, San Francisco, CA 94158, USA.
authors.forename: Joel
authors.initials: J
authors.lastname: Kramer
authors.affiliation: Community Hospital of the Monterrey Peninsula, 100 Clock Tower Pl, Ste 225, Carmel, CA 93921, USA.
authors.forename: Alejandro
authors.initials: A
authors.lastname: Centurion
authors.affiliation: Division of Neuro-oncology, Department of Neurology, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104, USA.
authors.forename: Josep
authors.initials: J
authors.lastname: Dalmau
authors.affiliation: Department of Pathology, University of California, San Francisco, 505 Parnassus Avenue, Suite M590, Box 0511, San Francisco, CA 94143, USA.
authors.forename: Eric
authors.initials: E
authors.lastname: Huang
authors.affiliation: Department of Pathology, University of California, San Francisco, 505 Parnassus Avenue, Suite M590, Box 0511, San Francisco, CA 94143, USA.
authors.forename: Jennifer A
authors.initials: JA
authors.lastname: Cotter
authors.affiliation: Memory and Aging Center, University of California, San Francisco, Sandler Neurosciences Centre, 675 Nelson Rising Lane, Suite 190, San Francisco, CA 94158, USA. Electronic address: michael.geschwind@ucsf.edu.
authors.forename: Michael D
authors.initials: MD
authors.lastname: Geschwind
mesh_terms: D000263:Adenylate Kinase / Q000276:immunology*; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000097:blood / Q000276:immunology / Q000473:pathology; D006801:Humans; D020363:Limbic Encephalitis / Q000097:blood / Q000276:immunology / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D013702:Temporal Lobe / Q000473:pathology
chemical_list: D001323:Autoantibodies; D000263:Adenylate Kinase; C118782:adenylate kinase 5
keywords: Adenylate kinase 5; Autoimmunity; Limbic encephalitis; Neuronal antibodies; Rapidly progressive dementia