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Epilepsy & behavior : E&B v.77, 2017년, pp.90 - 95   SCIE
본 등재정보는 저널의 등재정보를 참고하여 보여주는 베타서비스로 정확한 논문의 등재여부는 등재기관에 확인하시기 바랍니다.

Seizure semiology in leucine-rich glioma-inactivated protein 1 antibody-associated limbic encephalitis

Chen, Chao    (Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China   ); Wang, Xiu    (China National Clinical Research Center for Neurological Diseases, Beijing, China   ); Zhang, Chao    (China National Clinical Research Center for Neurological Diseases, Beijing, China   ); Cui, Tao    (Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China   ); Shi, Wei-Xiong    (Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China   ); Guan, Hong-Zhi    (Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China   ); Ren, Hai-Tao    (Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China   ); Shao, Xiao-Qiu    (Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China  );
  • 초록  

    Abstract 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. Highlights Based on semiology, LGI1 LE patient can be divided into three groups. Non-FBDS group manifested MTLE-like semiology with frequent and short duration attacks. In the FBDS+ group, the most common phenotype was FBDS followed by automatisms. Immunotherapy had favorable therapeutic effects on LGI1 LE.


  • 주제어

    Autoimmune encephalitis .   Faciobrachial dystonic seizure .   Semiology .   Mesial temporal lobe epilepsy.  

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