GABA <sub>A</sub> receptor and LGI1 antibody encephalitis in a patient with thymoma

Mateus Mistieri Simabukuro, Mar Petit‐Pedrol, Luiz Henrique Martins Castro, Ricardo Nitríni, Leandro Tavares Lucato, Antônio Alberto Zambon, Lis Gomes Silva, Gabriela Carneiro Rosa Fortes, Herval Ribeiro Soares Neto, Josep Dalmau

Neurology Neuroimmunology & Neuroinflammation · 2015 · 31 citations · 6 references

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Abstract

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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