LGI‐1 Encephalitis: A Comprehensive Multidisciplinary Paradigm for Diagnosis and Management

Abstract Introduction Leucine‐rich glioma‐inactivated 1 (LGI‐1) antibody encephalitis is a rare subtype of autoimmune limb encephalitis (ALE), which is marked by rapid neuropsychiatric decline. This report details a comprehensive approach to its diagnosis and management. Assessment In this case, a 68‐year‐old man presented with aggressive behaviors, cognitive decline, and seizure‐like episodes. Initial assessments suggested major neurocognitive disorder, with MMSE and MOCA scores of 20 and 17, respectively, indicating cognitive impairment. EEG confirmed episodes of facio‐brachial dystonic seizures. Further evaluation revealed hyponatremia, temporal lobe atrophy on MRI, and LGI1‐antibodies in the CSF. Treatment Initial treatments included steroids and IVIG, which did not result in significant improvement. However, after receiving two Rituximab trials, improvements in cognitive and behavioural outcomes were observed. Cognitive predictors of poor outcomes included older age, non‐response to initial therapies, and clinical relapses. Discussion This case underscores the complex presentation of LGI‐1 encephalitis, emphasizing the importance of thorough diagnostic evaluations and a multimodal treatment approach. The patient’s cognitive and behavioral improvements post‐treatment highlight the significance of timely and targeted interventions in managing this challenging autoimmune encephalitis. Long‐term follow‐up revealed sustained improvements, reinforcing the potential efficacy of a multidisciplinary treatment strategy.

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Publication Details

Journal
Alzheimer s & Dementia
Published
2024-12-01
DOI
https://doi.org/10.1002/alz.089908
Primary Topic
Autoimmune Neurological Disorders and Treatments
Type
article
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article

LGI‐1 Encephalitis: A Comprehensive Multidisciplinary Paradigm for Diagnosis and Management

Sarah Elmi, Keera Fishman, Emytis Tavakoli
Alzheimer s & Dementia
Autoimmune Neurological Disorders and Treatments
article

LGI‐1 Encephalitis: A Comprehensive Multidisciplinary Paradigm for Diagnosis and Management

Sarah Elmi, Keera Fishman, Emytis Tavakoli
article en

Abstract

Abstract Introduction Leucine‐rich glioma‐inactivated 1 (LGI‐1) antibody encephalitis is a rare subtype of autoimmune limb encephalitis (ALE), which is marked by rapid neuropsychiatric decline. This report details a comprehensive approach to its diagnosis and management. Assessment In this case, a 68‐year‐old man presented with aggressive behaviors, cognitive decline, and seizure‐like episodes. Initial assessments suggested major neurocognitive disorder, with MMSE and MOCA scores of 20 and 17, respectively, indicating cognitive impairment. EEG confirmed episodes of facio‐brachial dystonic seizures. Further evaluation revealed hyponatremia, temporal lobe atrophy on MRI, and LGI1‐antibodies in the CSF. Treatment Initial treatments included steroids and IVIG, which did not result in significant improvement. However, after receiving two Rituximab trials, improvements in cognitive and behavioural outcomes were observed. Cognitive predictors of poor outcomes included older age, non‐response to initial therapies, and clinical relapses. Discussion This case underscores the complex presentation of LGI‐1 encephalitis, emphasizing the importance of thorough diagnostic evaluations and a multimodal treatment approach. The patient’s cognitive and behavioral improvements post‐treatment highlight the significance of timely and targeted interventions in managing this challenging autoimmune encephalitis. Long‐term follow‐up revealed sustained improvements, reinforcing the potential efficacy of a multidisciplinary treatment strategy.

Alzheimer s & DementiaVol. 20(S5)
Ontario Shores Centre for Mental Health Sciences (CA), Baycrest Hospital (CA)
Openalex Percentile: Top 61%
Autoimmune Neurological Disorders and Treatments
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