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LGI1 Limbic Encephalitis — RACP Adult Medicine MCQ

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HardNeurologyLGI1 Limbic EncephalitisRACP Adult Medicine

A 65-year-old man presents with faciobrachial dystonic seizures (brief, frequent, stereotyped jerks of face and arm), progressive cognitive decline, and severe hyponatraemia (Na⁺ 118 mmol/L). MRI shows bilateral mesial temporal T2/FLAIR hyperintensity. CSF anti-LGI1 antibodies are positive. What is the most likely diagnosis?

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Correct answer: CLimbic encephalitis (LGI1)

Faciobrachial dystonic seizures (pathognomonic for LGI1 encephalitis), cognitive decline, severe hyponatraemia (SIADH-like), and mesial temporal MRI changes with positive anti-LGI1 antibodies is LGI1 limbic encephalitis. It is rarely paraneoplastic (<10%). Treatment is immunotherapy (corticosteroids, IVIG, PLEX). FBDS respond poorly to antiepileptics but well to immunotherapy.

Reference: Graus 2016 Autoimmune Encephalitis Criteria; eTG Neurology 2024