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
. 2014 Jan 8:4:tre-04-213-4821-1.
doi: 10.7916/D8MG7MFC.

Isolated Chorea Associated with LGI1 Antibody

Ritesh A Ramdhani  1 , Steven J Frucht  1
Affiliations

Isolated Chorea Associated with LGI1 Antibody

Ritesh A Ramdhani et al. Tremor Other Hyperkinet Mov (N Y). .

Abstract

Background: Leucine-rich glioma inactivated 1 (LGI1) antibody produces a syndrome of limbic encephalitis, hyponatremia, and facio-brachial dystonic seizures that is non-paraneoplastic and responsive to corticosteroids. Parkinsonism, tremor, and generalized chorea are rare manifestations of LGI1, but, when present, commonly accompany other signs of limbic encephalitis.

Case report: We present a case of LGI1-related isolated chorea in a 53-year-old Japanese male. His chorea responded to high-dose steroids, suggesting a potential role for this synaptic antibody in triggering chorea.

Discussion: This case highlights a new treatable etiology of chorea.

Keywords: LGI1 antibody; chorea; pulse steroids; reversible.

PubMed Disclaimer

Conflict of interest statement

Conflict of Interests: The authors report no conflict of interest.

References

    1. Lai M, Huijbers MG, Lancaster E, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series. Lancet Neurol. 2010;9:776–785. doi: 10.1016/S1474-4422(10)70137-X. - DOI - PMC - PubMed
    1. Irani SR, Michell AW, Lang B, et al. Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis. Ann Neurol. 2011;69:892–900. doi: 10.1002/ana.22307. - DOI - PubMed
    1. Rubio-Agusti I, Salavert M, Bataller L. Limbic encephalitis and related cortical syndromes. Curr Treat Options Neurol. 2013;15:169–184. doi: 10.1007/s11940-012-0212-7. - DOI - PubMed
    1. Thieben MJ, Lennon VA, Boeve BF, et al. Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody. Neurology. 2004;62:1177–1182. doi: 10.1212/01.WNL.0000122648.19196.02. - DOI - PubMed
    1. Vincent A, Buckley C, Lang B, et al. Clinical spectrum of voltage-gated potassium channel autoimmunity. Neurology. 2009;72:99. doi: 10.1212/01.wnl.0000339405.94708.8d. author reply 99–100. - DOI - PubMed

LinkOut - more resources