title: Cyclophosphamide for anti-GAD antibody-positive refractory status epilepticus.
abstract: Glutamic acid decarboxylase (GAD) is the enzyme which catalyzes the production of gamma aminobutyric acid (GABA), the main inhibitory neurotransmitter in the central nervous system (CNS). There is increasing evidence that severe GAD autoimmunity may be associated with refractory epilepsy. Immunomodulation and GABAergic drugs have been suggested as treatment options. We report here for the first time on a patient with sudden onset of refractory status epilepticus in the presence of strong intrathecal anti-GAD antibody synthesis who was successfully treated with cyclophosphamide, and give an overview of available data on epilepsy associated with GAD autoimmunity.
authors.affiliation: Department of Neurology, University of Erlangen, Erlangen, Germany. ines-christine.kanter@uk-erlangen.de
authors.forename: Ines C
authors.initials: IC
authors.lastname: Kanter
authors.forename: Hagen B
authors.initials: HB
authors.lastname: Huttner
authors.forename: Dimitre
authors.initials: D
authors.lastname: Staykov
authors.forename: Teresa
authors.initials: T
authors.lastname: Biermann
authors.forename: Tobias
authors.initials: T
authors.lastname: Struffert
authors.forename: Frank
authors.initials: F
authors.lastname: Kerling
authors.forename: Max-Josef
authors.initials: MJ
authors.lastname: Hilz
authors.forename: Peter D
authors.initials: PD
authors.lastname: Schellinger
authors.forename: Stefan
authors.initials: S
authors.lastname: Schwab
authors.forename: Jürgen
authors.initials: J
authors.lastname: Bardutzky
mesh_terms: D000328:Adult; D000888:Antibodies, Anti-Idiotypic / Q000096:biosynthesis / Q000187:drug effects* / Q000276:immunology; D000927:Anticonvulsants / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000276:immunology; D015551:Autoimmunity / Q000276:immunology; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004351:Drug Resistance; D017029:Epilepsy, Complex Partial / Q000188:drug therapy / Q000276:immunology; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D007278:Injections, Spinal; D008279:Magnetic Resonance Imaging; D013226:Status Epilepticus / Q000188:drug therapy* / Q000276:immunology* / Q000473:pathology; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: D000888:Antibodies, Anti-Idiotypic; D000927:Anticonvulsants; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D005968:Glutamate Decarboxylase