[LGI1 ENCEPHALITIS: THE FIRST HUNGARIAN PATIENT]

Ideggyogy Sz. 2015 Jul 30;68(7-8):279-85. doi: 10.18071/isz.68.0279.
[Article in Hungarian]

Abstract

In the recent years, it has been increasingly recognised that in a group of limbic encephalitis antibodies are directed against the scaffolding protein LGI1 (Leucine-rich glioma inactivated 1), which is part of the voltage gated potassium channel (VGKC) complex on neural synapses. Patients present with seizures and subacute history of neuropsychiatric symptoms, including psychosis and changes in memory, cognition, behaviour. Faciobrachial dystonic seizures can be observed, which are highly characteristic for LGI1 encephalitis. MRI shows medial temporal abnormalities in more than half of the cases. CSF evaluation is usually normal. Hyponatremia is frequently associated and may confuse the initial diagnosis. Early recognition and prompt initiation of immunotherapies are of great importance. The clinical improvements often correlate with the antibody levels. We present the case of a 64-year old man, who responded quickly to plasma exchange and major improvement was noted within few weeks.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Autoantibodies / blood*
  • Cognition
  • Diagnosis, Differential
  • Early Diagnosis
  • Humans
  • Hungary
  • Intracellular Signaling Peptides and Proteins
  • Limbic Encephalitis / complications
  • Limbic Encephalitis / diagnosis*
  • Limbic Encephalitis / metabolism
  • Limbic Encephalitis / psychology
  • Limbic Encephalitis / therapy*
  • Magnetic Resonance Imaging
  • Male
  • Memory
  • Middle Aged
  • Plasma Exchange*
  • Potassium Channels, Voltage-Gated / metabolism
  • Proteins / immunology*
  • Proteins / metabolism
  • Social Behavior
  • Temporal Lobe / metabolism
  • Temporal Lobe / pathology

Substances

  • Autoantibodies
  • Intracellular Signaling Peptides and Proteins
  • LGI1 protein, human
  • Potassium Channels, Voltage-Gated
  • Proteins