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Corticosteroids appear to be the ideal first-line agent in effectively treating LGI1-antibody encephalitis. Evidence for optimal additional therapies is awaited.
Autoantibodies against leucine glioma inactivated 1 (LGI1) are well recognised in elderly men without tumours who present with frequent, focal seizures and a hippocampal-centric pattern of cognitive impairment.1 These patients often progress over more than 3 months and show little evidence of inflammation on routine CSF testing, defying diagnostic criteria for autoimmune encephalitis. Furthermore, as the LGI1-antibodies are of the ‘non-inflammatory’ IgG4 subclass, several first principles collectively question a rationale for immunotherapy administration in this cohort.
Yet, this form of autoantibody-mediated central nervous system disease is clearly, and often exquisitely, sensitive to immunotherapies. We have witnessed patients whose seizure frequency plummets from 100/day to nil after just three doses of corticosteroids. Larger studies have confirmed time-dependent immunotherapy sensitivity. For example, cessation of faciobrachial dystonic seizures (the most characteristic semiology in these patients) was observed in 75/85 (88%) patients 90 days after immunotherapy administration: an effect most marked in those who received early immunotherapies.2 However, the question of which immunotherapies to administer remains unanswered. The …
Contributors SRI wrote and edited the manuscript.
Funding SRI is supported by the Medical Research Council Fellowship (MR/V007173/1), Wellcome Trust (104079/Z/14/Z), BMA Research Grants—2013 Vera Down grant and 2017 Margaret Temple, Epilepsy Research UK (P1201) and by the Fulbright UK-US commission (MS-Research Society Award). SB is supported by the Wellcome Trust. The work is supported by the National Institute for Health Research (NIHR) Oxford Biomedical Research Centre (BRC). The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health. For the purpose of Open Access, the author has applied a CC BY public copyright licence to any Author Accepted Manuscript version arising from this submission.
Competing interests SRI is a co-applicant and receive royalties on patent application WO/2010/046716 entitled ‘Neurological Autoimmune Disorders’. The patent has been licensed to Euroimmun AG for the development of assays for LGI1 and other VGKC-complex antibodies. SRI is inventor on ‘A Diagnostic Strategy to improve specificity of CASPR2 antibody detection.’ Ref. JA94536P. SRI has received honoraria from UCB, Immunovant, Roche, Cerebral Therapeutics, MedImmun, ADC therapeutics, Medlink Neurology and research support from CSL Behring, UCB and ONO Pharma.
Provenance and peer review Commissioned; internally peer reviewed.