Natural course of LGI1 encephalitis: 3-5 years of follow-up without immunotherapy

Monika Szots, Annamaria Marton, Ferenc Kover, Tunde Kiss, Timea Berki, Ferenc Nagy, Zsolt Illes

Research output: Contribution to journalJournal articleResearchpeer-review

Abstract

Antibodies against LGI1 (leucin-rich glioma-inactivated 1 protein) are associated with limbic encephalitis (LE), which is characterized by a favorable outcome following immunotherapy. Here, we present two cases, where antibodies against LGI1 were detected in the sera 36 and 53 months after acute LE, respectively, and none of the patients received immunotherapy. LE showed characteristics of LGI1 encephalitis in both cases, including low sodium content in the sera; disorientation, hallucination, short-term memory loss; and epileptic seizures. One patient had faciobrachial tonic seizures. MRI indicated bilateral inflammation of the hippocampus in one case. We reviewed longitudinal clinical and MRI data covering 53 and 36 months after LE without immunotherapy, respectively. Both patients became seizure-free and spontaneously recovered with mild/moderate cognitive impairment. No relapses have been observed. Follow-up brain MRI indicated early hippocampal sclerosis and global brain atrophy in one case characterized by more pronounced cognitive deficit. Memory and verbal fluency were affected most during the natural course of LGI1 encephalitis. LGI1 encephalitis had a monophasic course and spontaneously improved, suggesting that a relatively benign natural course may contribute to the favorable outcome observed after immunotherapy. Our data also indicate that LGI1 antibodies can be present in the sera without clinical disease activity.

Original languageEnglish
JournalJournal of the Neurological Sciences
Volume343
Issue number1-2
Pages (from-to)198-202
Number of pages5
ISSN0022-510X
DOIs
Publication statusPublished - 2014

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