Antibody-LGI 1 autoimmune encephalitis manifesting as rapidly progressive dementia and hyponatremia: a case report and literature review

BMC Neurology

Junliang Yuan¹  Wenli Hu¹  Xuanting Li¹  Lei Liu² 

[1] Department of Neurology, Beijing Chaoyang Hospital, Capital Medical University;Department of Neurology, Beijing Tongren Hospital, Capital Medical University;

关键词: Autoimmune encephalitis;    Limbic encephalitis;    Leucine-rich glioma inactivated 1;    Cognitive impairment;    Hyponatremia;    Arterial spin labeling;   

DOI  :  10.1186/s12883-019-1251-4

来源: DOAJ

【 摘 要 】

Abstract Background Anti leucine-rich glioma inactivated 1 (LGI1) encephalitis is a rare autoimmune encephalitis (AE), characterized by acute or subacute cognitive impairment, faciobrachial dystonic seizures, psychiatric disturbances and hyponatremia. Antibody-LGI 1 autoimmune encephalitis (anti-LGI1 AE) has increasingly been recognized as a primary autoimmune disorder with favorable prognosis and response to treatment. Case presentation Herein, we reported a male patient presenting as rapidly progressive dementia and hyponatremia. He had antibodies targeting LGI1 both in the cerebrospinal fluid and serum, which demonstrated the diagnosis of typical anti-LGI1 AE. The scores of Mini-Mental State Examination and Montreal Cognitive Assessment were 19/30 and 15/30, respectively. Cranial magnetic resonance images indicated hyperintensities in bilateral hippocampus. The findings of brain arterial spin labeling and Fluorine-18-fluorodeoxyglucose positron emission tomography showed no abnormal perfusion/metabolism. After the combined treatment of intravenous immunoglobulin and glucocorticoid, the patient’s clinical symptoms improved obviously. Conclusions This case raises the awareness that a rapid progressive dementia with predominant memory deficits could be induced by immunoreactions against LGI1. The better recognition will be great importance for the early diagnosis, essential treatment, even a better prognosis.

【 授权许可】

Unknown