Jerking & confused: Leucine-rich glioma inactivated 1 receptor encephalitis

J Neuroimmunol. 2015 Dec 15:289:84-6. doi: 10.1016/j.jneuroim.2015.10.010. Epub 2015 Oct 21.

Abstract

This is a case of autoimmune encephalitis with features of faciobrachial dystonic seizures (FBDS) pathognomonic for Leucine Rich Glioma inactivated (LGI)1 antibody encephalitis. This voltage-gated potassium channel complex encephalitis is marked by rapid onset dementia, FBDS and hyponatremia, which is sensitive to management with immunotherapy including steroids, IVIG and other agents. In this case report we review the clinical features, imaging and management of this condition.

Keywords: Faciobrachial dystonic seizures; LGI1 encephalitis; Limbic encephalitis; Paraneoplastic disease; Steroid sensitive encephalopathy.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Aged
  • Amygdala / diagnostic imaging
  • Amygdala / drug effects
  • Amygdala / metabolism
  • Antibodies / blood*
  • Cognition Disorders / drug therapy
  • Cognition Disorders / etiology
  • Encephalitis / blood*
  • Encephalitis / complications
  • Encephalitis / immunology*
  • Encephalitis / therapy
  • Humans
  • Intracellular Signaling Peptides and Proteins
  • Magnetic Resonance Imaging
  • Male
  • Positron-Emission Tomography
  • Proteins / immunology*
  • Seizures / drug therapy
  • Seizures / etiology

Substances

  • Adrenal Cortex Hormones
  • Antibodies
  • Intracellular Signaling Peptides and Proteins
  • LGI1 protein, human
  • Proteins