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Case Reports
. 2019 Oct 9:9.
doi: 10.7916/tohm.v0.663. eCollection 2019.

Leucine-Rich Glioma-Inactivated 1 Encephalitis: Broadening the Sphere

Amine Rachdi  1 , Julia Dupouy  2 , Marie Benaiteau  2 , Chloé Bost  3 , Marion Simonetta Moreau  2 , Christine Brefel Courbon  2 , Olivier Rascol  1   2 , Fabienne Ory Magne  2
Affiliations
Case Reports

Leucine-Rich Glioma-Inactivated 1 Encephalitis: Broadening the Sphere

Amine Rachdi et al. Tremor Other Hyperkinet Mov (N Y). .

Abstract

Background: Leucine-rich glioma-inactivated 1 (LGI1) encephalitis is a rare entity. Its typical features are seizures, faciobrachial dystonic seizures (FBDS), cognitive impairment, and personality changes.

Case report: We report the case of a 66-year-old man with an unusual presentation, consisting of two types of FBDS, one starting in the foot and the other consisting of asynchronous myoclonic and dystonic jerks of the face triggered by noise and chin stimulation. The patient displayed no personality changes or cognitive impairment.

Discussion: LGI1 encephalitis is a heterogeneous disease. Many different forms of FBDS may be observed, and these seizures can be the only symptom. This type of encephalitis should be suspected in presenting very frequent episodic events with dystonic features, regardless of the part of the body affected.

Keywords: Automimmune encephalitis; LGI1 encephalitis; dystonia; seizure-like.

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Conflict of interest statement

Funding: None. Conflicts of Interest: The authors report no conflict of interest. Ethics Statement: All patients who appear on video have provided written informed consent; authorization for the videotaping and for publication of the videotape was also obtained.

References

    1. Balint B, Vincent A, Meinck H-M, Irani SR, Bhatia KP. Movement disorders with neuronal antibodies: syndromic approach, genetic parallels and pathophysiology. Brain 2018;141(1):13–36. doi: 10.1093/brain/awx189 - DOI - PMC - PubMed
    1. Binks SNM, Klein CJ, Waters P, Pittock SJ, Irani SR. LGI1, CASPR2 and related antibodies: a molecular evolution of the phenotypes. J Neurol Neurosurg Psychiatry 2018;89(5):526–534. doi: 10.1136/jnnp-2017-315720 - DOI - PMC - PubMed
    1. Irani SR, Michell AW, Lang B, Pettingill P, Waters P, Johnson MR, et al. . Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis. Ann Neurol 2011;69(5):892–900. doi: 10.1002/ana.22307 - DOI - PubMed
    1. Irani SR, Stagg CJ, Schott JM, Rosenthal CR, Schneider SA, Pettingill P, et al. . Faciobrachial dystonic seizures: the influence of immunotherapy on seizure control and prevention of cognitive impairment in a broadening phenotype. Brain 2013;136(Pt 10):3151–3162. doi: 10.1093/brain/awt212 - DOI - PubMed
    1. Damato V, Balint B, Kienzler AK, Irani SR. The clinical features, underlying immunology, and treatment of autoantibody-mediated movement disorders. Mov Disord 2018;33(9):1376–1389. doi: 10.1002/mds.27446 [Epub 2018 Sep 14]. - DOI - PMC - PubMed

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