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

  • Amine Rachdi
  • Julia Dupouy
  • Marie Benaiteau
  • Chloé Bost
  • Marion Simonetta Moreau
  • Christine Brefel Courbon
  • Olivier Rascol
  • Fabienne Ory Magne


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, LGI1 atypical presentation, dystonia, seizure-like

Citation: Rachdi A, Dupouy J, Beneteau M, Moreau MS, Courbon CB, Rascol O, et al. Leucine-rich glioma-inactivated 1 encephalitis: Broadening the sphere. Tremor Other Hyperkinet Mov. 2019; 9. doi: 10.7916/tohm.v0.663