Clinical analysis of patients with anti-leucine-rich glioma-inactivated 1 antibody-associated limbic encephalitis

Objective To investigate the clinical characteristics of anti-leucine-rich glioma-inactivated 1 (LGI1) antibody-associated encephalitis. Methods and Results From June 2016 to October 2017, a total of 7 patients with anti-LGI1 antibody-associated limbic encephalitis were diagnosed and treated, with a...

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Published inZhongguo xian dai shen jing ji bing za zhi Vol. 19; no. 4; pp. 271 - 276
Main Authors Jia-qian TANG, Li XU, Zhi-yao YU, Lei HUANG, Fang LIU
Format Journal Article
LanguageEnglish
Published Tianjin Huanhu Hospital 01.04.2019
Subjects
Antibodies
Epilepsy
Immunotherapy
Limbic encephalitis
Memory disorders
Tumor suppressor proteins
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Summary:Objective To investigate the clinical characteristics of anti-leucine-rich glioma-inactivated 1 (LGI1) antibody-associated encephalitis. Methods and Results From June 2016 to October 2017, a total of 7 patients with anti-LGI1 antibody-associated limbic encephalitis were diagnosed and treated, with an average age at onset (48.29 ± 15.09) years. Patients presented acute (4 cases) or subacute onset (3 cases), with seizures and memory dysfunction as the main manifestations. It may be accompanied by faciobrachial dystonic seizures (FBDS, 5 cases), mental and behavioral abnormalities or personality changes (4 cases), or even combined with intractable hyponatremia (2 cases) or thymoma (one case). Serum anti-LGI1 antibody tests showed positive results in 7 cases, and cerebrospinal fluid (CSF) anti-LGI1 antibody tests showed positive results in 6 cases. MRI showed unilateral or bilateral medial temporal lobe abnormal signals (6 cases), and EEG showed continuous spike-slow waves or slow waves (one case). High-dose glucocorticoid sequential therapy was effective in 6 cases. During the follow-up period, 2 cases were lost, and the other 5 cases presented long-term memory disorder (3 cases) or long-term and short-term memory disorders (2 cases). Among them, one case relapsed 6 months after discharge. Conclusions According to specific clinical manifestations of patients (such as onset of FBDS, memory disorders, etc.), combined with imaging and CSF examination results, this disease can be clearly diagnosed. Immunosuppressive agents can effectively improve the clinical symptoms and prognosis. DOI: 10.3969/j.issn.1672-6731.2019.04.010
ISSN:1672-6731