title: Non-paraneoplastic limbic encephalitis associated with anti-glutamic acid decarboxylase antibodies.
abstract: Limbic encephalitis (LE) is a neurological syndrome that may present in association with cancer, infection, or as an isolate clinical condition often accompanying autoimmune disorders. Here we have characterized the clinical and laboratory features of two patients presenting with subacute onset, and chronic evolution, of anterograde amnesia and drug-resistant epilepsy associated with thyroid autoimmunity and in absence of tumoral pathology despite long follow-up. Antibodies against onconeural antigens, voltage gated potassium channel and glutamate receptors, which may accompany paraneoplastic as well as non-paraneoplastic LE, were negative. However, biochemical studies showed high titers, and sustained intrathecal synthesis, of antibodies directed against neuronal glutamic acid decarboxylase (GAD). In one patient, plasma exchange determined a dramatic improvement of the neurological deficits along with the decrease of autoantibodies.
authors.affiliation: Department of Neurological and Psychiatric Sciences, University of Firenze, Italy. masa@unifi.it
authors.forename: Sabrina
authors.initials: S
authors.lastname: Matà
authors.forename: Gian Carlo
authors.initials: GC
authors.lastname: Muscas
authors.forename: Ilaria
authors.initials: I
authors.lastname: Naldi
authors.forename: Eleonora
authors.initials: E
authors.lastname: Rosati
authors.forename: Sergio
authors.initials: S
authors.lastname: Paladini
authors.forename: Barbara
authors.initials: B
authors.lastname: Cruciatti
authors.forename: Francesca
authors.initials: F
authors.lastname: Bisulli
authors.forename: Marco
authors.initials: M
authors.lastname: Paganini
authors.forename: Gustavo
authors.initials: G
authors.lastname: Mazzi
authors.forename: Sandro
authors.initials: S
authors.lastname: Sorbi
authors.forename: Paolo
authors.initials: P
authors.lastname: Tinuper
mesh_terms: D000328:Adult; D000927:Anticonvulsants / Q000627:therapeutic use; D001323:Autoantibodies / Q000134:cerebrospinal fluid / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology*; D001921:Brain / Q000473:pathology*; D004279:DNA, Viral / Q000134:cerebrospinal fluid; D003922:Diabetes Mellitus, Type 1; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology*; D015654:Herpesvirus 6, Human; D006801:Humans; D020363:Limbic Encephalitis / Q000276:immunology* / Q000473:pathology* / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D010956:Plasmapheresis; D019349:Roseolovirus Infections / Q000134:cerebrospinal fluid / Q000150:complications; D012640:Seizures / Q000209:etiology
chemical_list: D000927:Anticonvulsants; D001323:Autoantibodies; D001324:Autoantigens; D004279:DNA, Viral; D005968:Glutamate Decarboxylase