Encephalitis with leucine-rich glioma-inactivated-1 (LGI1) immunoglobulin G (IgG) antibodies classically presents with cognitive impairment and characteristic faciobrachial dystonic seizures.1 In a murine model, human LGI1 IgG caused reduction of Kv1.1 channels and α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor receptors resulting in neuronal hyperexcitability indicating probable pathogenicity of LGI1 antibodies.2 LGI1 autoimmunity is associated with malignancy in less than 10% of cases, including small cell lung cancer, prostate and colon cancer, squamous cell skin carcinoma, and neuroendocrine pancreatic cancer.3,4 We present a case of LGI1 encephalitis only partially responsive to immunotherapy with eventual complete resolution after resection of a squamous cell lung carcinoma.
Acknowledgment
The authors express our appreciation to Sofia Akrivou and Dr. Harry Alexopoulos in Dr. Dalakas's neuroimmunology laboratory for performing the tissue immunostaining. Publication was made possible in part by support from the Thomas Jefferson University Open Access Fund.
Footnotes
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The Article Processing Charge was funded by the authors.
- Received December 12, 2019.
- Accepted in final form September 11, 2020.
- Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
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