Non-paraneoplastic limbic encephalitis associated with anti-glutamic acid decarboxylase antibodies.
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.
Matà
Sabrina
S
Department of Neurological and Psychiatric Sciences, University of Firenze, Italy. masa@unifi.it
Muscas
Gian Carlo
GC
Naldi
Ilaria
I
Rosati
Eleonora
E
Paladini
Sergio
S
Cruciatti
Barbara
B
Bisulli
Francesca
F
Paganini
Marco
M
Mazzi
Gustavo
G
Sorbi
Sandro
S
Tinuper
Paolo
P
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
D000927:Anticonvulsants; D001323:Autoantibodies; D001324:Autoantigens; D004279:DNA, Viral; D005968:Glutamate Decarboxylase