Lehong Gao1, Aihua Liu1, Shuqin Zhan1, Li Wang1, Liping Li1, Le Guan2, Xin Zhao1, Xiating Zhang1, Yuping Wang3. 1. Department of Neurology, Xuanwu Hospital, Capital Medical University, The Beijing Key Laboratory of Neuromodulation, Beijing, China. 2. Department of Nuclear Medicine, Xuanwu Hospital, Capital Medical University, Beijing, China. 3. Department of Neurology, Xuanwu Hospital, Capital Medical University, The Beijing Key Laboratory of Neuromodulation, Beijing, China. Electronic address: doctorwangyuping@163.com.
Abstract
OBJECTIVE: Autoimmune encephalitis associated with antibodies to leucine-rich glioma inactivated 1 (LGI1) has recently been identified and is characterized by an acute to subacute onset of cognitive impairment and convulsion, faciobrachial dystonic seizures (FBDSs), and psychiatric disturbances. This study analyzed the clinical characteristics and outcomes of 10 patients with LGI1 antibody encephalitis in order to further understand this disease and to improve its therapeutic strategies. METHODS: Between January 2013 and March 2015, we identified 10 patients with LGI1 antibody encephalitis. We retrospectively analyzed the clinical details, laboratory results, electrophysiological and imaging findings, and the treatment outcomes. RESULTS: All patients tested had LGI1 antibodies. Immunotherapy was effective in all patients. Seizures in patients with FBDS showed a poor response to antiepileptic drugs. Two patients examined by magnetoencephalogram (MEG) during the acute disease phase showed a small quantity of spike-wave dipoles in the temporal lobe close to the lateral fissure and insular lobe. CONCLUSION: Patients with LGI1 antibody encephalitis responded well to immunotherapy. We speculate that FBDS is likely a form of insular epilepsy.
OBJECTIVE:Autoimmune encephalitis associated with antibodies to leucine-rich glioma inactivated 1 (LGI1) has recently been identified and is characterized by an acute to subacute onset of cognitive impairment and convulsion, faciobrachial dystonic seizures (FBDSs), and psychiatric disturbances. This study analyzed the clinical characteristics and outcomes of 10 patients with LGI1 antibody encephalitis in order to further understand this disease and to improve its therapeutic strategies. METHODS: Between January 2013 and March 2015, we identified 10 patients with LGI1 antibody encephalitis. We retrospectively analyzed the clinical details, laboratory results, electrophysiological and imaging findings, and the treatment outcomes. RESULTS: All patients tested had LGI1 antibodies. Immunotherapy was effective in all patients. Seizures in patients with FBDS showed a poor response to antiepileptic drugs. Two patients examined by magnetoencephalogram (MEG) during the acute disease phase showed a small quantity of spike-wave dipoles in the temporal lobe close to the lateral fissure and insular lobe. CONCLUSION:Patients with LGI1 antibody encephalitis responded well to immunotherapy. We speculate that FBDS is likely a form of insular epilepsy.
Authors: Claude Steriade; Lara Jehi; Balu Krishnan; Marcia Morita-Sherman; Ahsan N V Moosa; Stephen Hantus; Patrick Chauvel Journal: Clin Neurophysiol Date: 2020-05-11 Impact factor: 3.708
Authors: Gabriela Dumitrita Stanciu; Veronica Bild; Daniela Carmen Ababei; Razvan Nicolae Rusu; Sorin Ioan Beschea Chiriac; Elena Rezuş; Andrei Luca Journal: Int J Mol Sci Date: 2019-09-13 Impact factor: 5.923