GABAA receptor and LGI1 antibody encephalitis in a patient with thymoma
2015
Autoimmune encephalitis related to antibodies against neuronal cell surface and synaptic proteins is a new category of disorders in which the targets are well-known proteins and receptors involved in synaptic transmission and neuronal excitability. GABAA receptor is one of the latest identified antigens within this category.1 High-titer serum and CSF GABAA receptor antibodies were recently reported in 6 patients with autoimmune encephalitis associated with seizures or status epilepticus, 4 of them requiring pharmacologic-induced coma. Patients' brain MRIs showed characteristic multiple cortical and subcortical abnormalities with fluid-attenuated inversion recovery (FLAIR)/T2 hyperintensity. Antibodies to LGI1 are associated with limbic encephalitis previously attributed to voltage-gated potassium channels (VGKC).2 Coexistence of these antibodies is rare and intriguing.
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