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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. |
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amnesia amnesia,anterograde autoantibodies autoimmune disease cerebrospinal fluid,oligoclonal IgG in encephalitis encephalitis,autoimmune glutamic acid decarboxylase,antibody hippocampus hypothyroidism limbic encephalitis memory,impairment of MRI,abnormal plasmapheresis seizure seizure,intractable seizure,psychomotor-temporal lobe symmetric brain lesions temporal lobe temporal lobe,lesion temporal lobe,lesion,bilateral thyroid function tests thyroiditis treatment of neurologic disorder
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