Abstract

Background: Cognitive impairment is a common manifestation of anti-LGI1 encephalitis and is typically defined as prominent memory deficits. We frequently encounter frontal cognitive-behavioural deficits when evaluating these patients, but this has yet to be well described in the literature. Methods: Patients with anti-LGI1 encephalitis were retrospectively identified from three tertiary centres in Toronto, Ontario between 2013 and 2022. Their medical records were evaluated and frontal features were categorized based on diagnostic criteria for behavioural variant frontotemporal dementia (bvFTD). Results: Nineteen patients were identified (median age 60 years [range 18–84]; 10 [52.6%] male). Eighteen (94.7%) had frontal cognitive-behavioural symptoms. Two developed these symptoms during treatment with steroids and were excluded from further analysis. The remaining 16 presented with behavioural disinhibition (n=13), apathy or inertia (n=6), perseverative, stereotyped or compulsive/ritualistic behaviours (n=6), hyperorality and dietary changes (n=4), a neuropsychological profile with predominant deficits in executive tasks (n=4), and loss of sympathy or empathy (n=4). Nine (47.3%) met diagnostic criteria for possible bvFTD. Anterograde memory impairment was common (n=14). Of the 16 patients with frontal features, 6 had faciobrachial dystonic seizures. Conclusions: Patients with anti-LGI1 encephalitis exhibit frontal cognitive-behavioural symptoms in addition to memory impairment. Clinicians should consider anti-LGI1 encephalitis in the differential diagnosis of bvFTD.

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