Seizure semiology in leucine-rich glioma-inactivated protein 1 antibody-associated limbic encephalitis.
OBJECTIVE
The objective of this study was to advance the characterization of seizure semiology in leucine-rich glioma-inactivated protein 1 (LGI1) antibody-associated limbic encephalitis (LE).


METHODS
Eighteen patients diagnosed with LGI1 LE were identified. Seizure semiology, demographic features, MRI and fluorodeoxyglucose positron emission tomography (FDG-PET), electroencephalograms, and outcomes following immunotherapy were evaluated.


RESULTS
Patients were divided into the following groups based on seizure semiology: faciobrachial dystonic seizure only (FBDS-only, n=4), epileptic seizure without FBDS (Non-FBDS, n=6), and FBDS plus epileptic seizure (FBDS+, n=8). In the group with Non-FBDS, the majority of patients (5/6) manifested mesial temporal lobe epilepsy (MTLE) like semiology (i.e., fear, epigastric rising, staring, and automatisms) with a frequency of 7±5 times per day and a duration of 15.3±14.3s. In the group with FBDS+, the distinctive symptom was FBDS followed by epileptic events, especially automatisms (7/8), with a frequency of 16±12 times per day and a duration of 13.0±8.0s. In these cases, 67% and 50% of the patients showed abnormalities on MRI and FDG-PET, respectively, and the mesial temporal lobe structures were most often involved. Ictal discharges were observed in 0/4, 6/6, and 8/8 of the patients in the groups with FBDS only, Non-FBDS, and FBDS+, respectively. The temporal lobe was mainly affected. Immunotherapy had favorable therapeutic effects.


SIGNIFICANCE
The LGI1 LE should be considered as one disease syndrome with a series of clinical manifestation. Identifying types of unique semiology features will facilitate the early diagnosis and the timely initiation of immunotherapy.
['Chen|Chao|C|', 'Wang|Xiu|X|', 'Zhang|Chao|C|', 'Cui|Tao|T|', 'Shi|Wei-Xiong|WX|', 'Guan|Hong-Zhi|HZ|', 'Ren|Hai-Tao|HT|', 'Shao|Xiao-Qiu|XQ|']
D000328:Adult D000368:Aged D001323:Autoantibodies D004569:Electroencephalography D005260:Female D006801:Humans D007167:Immunotherapy D047908:Intracellular Signaling Peptides and Proteins D020363:Limbic Encephalitis D008279:Magnetic Resonance Imaging D008297:Male D008875:Middle Aged D011506:Proteins D012640:Seizures D016896:Treatment Outcome
Semiology Faciobrachial dystonic seizure Mesial temporal lobe epilepsy Autoimmune encephalitis