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 o...

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Bibliographic Details
Published in:Journal of neuroimmunology 2008-08, Vol.199 (1), p.155-159
Main Authors: Matà, Sabrina, Muscas, Gian Carlo, Naldi, Ilaria, Rosati, Eleonora, Paladini, Sergio, Cruciatti, Barbara, Bisulli, Francesca, Paganini, Marco, Mazzi, Gustavo, Sorbi, Sandro, Tinuper, Paolo
Format: Article
Language:English
Subjects:
Adult
Allergy and Immunology
Anti-GAD antibodies
Anticonvulsants - therapeutic use
Autoantibodies - cerebrospinal fluid
Autoantibodies - immunology
Autoantigens - immunology
Brain - pathology
Diabetes Mellitus, Type 1
DNA, Viral - cerebrospinal fluid
Female
Glutamate Decarboxylase - immunology
Hashimoto's encephalopathy
Herpesvirus 6, Human
Human herpesvirus 6
Humans
Intrathecal synthesis
Limbic encephalitis
Limbic Encephalitis - immunology
Limbic Encephalitis - pathology
Limbic Encephalitis - therapy
Magnetic Resonance Imaging
Middle Aged
Neurology
Plasmapheresis
Roseolovirus Infections - cerebrospinal fluid
Roseolovirus Infections - complications
Seizures - etiology
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Summary: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.
ISSN:0165-5728
1872-8421
DOI:10.1016/j.jneuroim.2008.05.015