Cyclophosphamide for anti-GAD antibody-positive refractory status epilepticus.
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.
Kanter
Ines C
IC
Department of Neurology, University of Erlangen, Erlangen, Germany. ines-christine.kanter@uk-erlangen.de
Huttner
Hagen B
HB
Staykov
Dimitre
D
Biermann
Teresa
T
Struffert
Tobias
T
Kerling
Frank
F
Hilz
Max-Josef
MJ
Schellinger
Peter D
PD
Schwab
Stefan
S
Bardutzky
Jürgen
J
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
D000888:Antibodies, Anti-Idiotypic; D000927:Anticonvulsants; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D005968:Glutamate Decarboxylase