Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2018 Jul 6;18(1):96.
doi: 10.1186/s12883-018-1099-z.

Clinical characteristics and short-term prognosis of LGI1 antibody encephalitis: a retrospective case study

Weishuai Li  1 , Si Wu  1 , Qingping Meng  1 , Xiaotian Zhang  1 , Yang Guo  1 , Lin Cong  1 , Shuyan Cong  1 , Dongming Zheng  2
Affiliations

Clinical characteristics and short-term prognosis of LGI1 antibody encephalitis: a retrospective case study

Weishuai Li et al. BMC Neurol. .

Abstract

Background: Recently, most reports of Leucine-rich glioma-inactivated 1 (LGI1) antibody encephalitis are from Europe and the US, while the short term outcome and clinical characteristics of Chinese patients are rarely reported,we study the clinical manifestations, laboratory results and brain magnetic resonance images (MRI) of eight patients who were recently diagnosed with LGI1 antibody encephalitis in our hospital to improve the awareness and knowledge of this disease.

Methods: Eight patients (five males and three females; mean age, 63.4) with LGI1 antibody encephalitis who were diagnosed and treated in the Department of Neurology of Shengjing Hospital of China Medical University from September 2016 to June 2017 were recruited for the current study. Their general information, clinical manifestations, treatment regimens, and short-term prognoses were retrospectively analyzed, as were the results from MRI and laboratory findings.

Results: Overall, patient symptoms included cognitive impairment, which manifested primarily as memory deficits (8/8), seizures (including faciobrachial dystonic seizure, (FBDS)) (8/8), psychiatric and behavioral disorders (7/8), sleep disorders (4/8), and autonomic abnormalities (3/8). Five patients also had abnormal findings on brain MRI, mainly involving the hippocampus, basal ganglia and insula. Hyponatremia occurred in six cases. All patients tested positive for LGI1 antibodies in their serum/cerebrospinal fluid (CSF)and patients were negative for tumors. Symptoms rapidly improved after treatment with immunoglobulin and/or steroid therapy. The patients were followed up for 4-13 months after discharge, and two patients relapsed.

Conclusion: Primary symptoms of LGI1 antibody encephalitis include memory impairments, seizures, FBDS, and mental and behavioral abnormalities. Increased titers of LGI1 antibodies are also present in the serum/CSF of patients. Patients often have hyponatremia, and MRIs show abnormalities in various brain regions. Finally, immunotherapy shows good efficacy and positive benefits, although patients may relapse in the short-term.

Keywords: Autoimmune encephalitis; Cognitive function; Epilepsy; Magnetic resonance imaging.

PubMed Disclaimer

Conflict of interest statement

Ethics approval and consent to participate

This study was approved by the Ethics Committee of Affiliated Shengjing Hospital of China Medical University. All patients or the patient’s next-of-kin provided written informed consent to participate if a patient could not sign due to disability. And this was also approved by the ethics committee. A copy of the written consent is available for review by the Editor of this journal.

Consent for publication

Written informed consent was obtained from the patients for publication of this research and any accompanying images or from the patient’s next-of-kin.

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
MRI images of five patients with LGI1 antibody encephalitis. a FLAIR sequences and high T2 signal changes in the right insula in case 1; b FLAIR sequences and high T2 signal changes in the bilateral hippocampus in case 3; c FLAIR sequences and high T2 signal changes in the right hippocampus in case 5; D1 Abnormal signals in the bilateral caudate nucleus and putamen in case 4 at admission; MRI follow-up after 1 month (D2) and 2 months (D3) showed that the lesion signal intensity changed to a high T1 signal; (E1) Abnormal signals in bilateral caudate nucleus in case 7 at admission; E2 MRI of case 7 after 5 months. Abbreviations: MRI, magnetic resonance imaging; FLAIR, fluid-attenuated inversion recovery
See this image and copyright information in PMC

References

    1. ELancaster , J Dalmau Neuronalautoantigens--pathogenesis, associated disorders and antibody testing. Nat Rev Neurol. 2012;8:380–390. doi: 10.1038/nrneurol.2012.99. - DOI - PMC - PubMed
    1. IraniSR AS, Waters P, Kleopa KA, Pettingill P, Zuliani L, Peles E, Buckley C, Lang B, Vincent A. Antibodies to Kv1 potassiumchannel-complex proteins leucine-rich, glioma inactivated 1 protein andcontactin-associated protein-2 in limbic encephalitis, Morvan’s syndrome andacquired neuromyotonia. Brain. 2010;133:2734–2748. doi: 10.1093/brain/awq213. - DOI - PMC - PubMed
    1. Irani SR, Gelfand JM, Al-Diwani A, Vincent A. Cell-surface central nervous system autoantibodies:clinical relevance and emerging paradigms. Ann Neurol. 2014;76:168–184. doi: 10.1002/ana.24200. - DOI - PMC - PubMed
    1. Asztely F, Kumlien E. The diagnosis and treatment of limbic encephalitis. Acta Neurol Scand. 2012;126:365–375. doi: 10.1111/j.1600-0404.2012.01691.x. - DOI - PubMed
    1. Lai M, Huijbers MG, Lancaster E, Graus F, Bataller L, Balice-Gordon R, Cowell JK, Dalmau J. Investigation of LGI1 as theantigen in limbic encephalitis previously attributed to potassiumchannels: a case series. Lancet Neurol. 2010;9:776–785. doi: 10.1016/S1474-4422(10)70137-X. - DOI - PMC - PubMed

LinkOut - more resources