Clinical features and outcomes of leucine-rich glioma-inactivated protein 1 and contactin protein-like 2 antibody-associated autoimmune encephalitis in a Chinese cohort

J Clin Neurosci. 2022 Jul:101:137-143. doi: 10.1016/j.jocn.2022.05.007. Epub 2022 May 18.

Abstract

The present study aimed to investigate the clinical manifestations, epidemiological characteristics, and outcomes of Chinese patients with voltage-gated potassium channel complex (VGKC) antibody-associated encephalitis. Patients diagnosed with VGKC antibody-associated encephalitis at our institution between January 2016 and December 2020 were included in this study. We retrospectively evaluated their clinical features, auxiliary examination results, treatments details, long-term outcomes, and risk factors for poor outcome. Of the 91 included patients, 61 (67.78%) were men and 30 (32.97%) were women. The most common clinical symptoms were seizures (n = 63, 69.23%), memory deficits (n = 62, 68.13%), mental behavioral disorders (n = 29, 31.87%), and hyponatremia (n = 57, 62.64%). Although patients with anti- leucine-rich glioma-inactivated 1 (LGI1) (n = 76) and anti- contactin-associated protein-like 2 (CASPR2) encephalitis (n = 15) had similar clinical manifestations, the former were more diverse. In total, 86 (94.51%) patients were treated with immunotherapy. Over a median follow-up period of 25 months, there were no mortalities and 14 (15.38%) patients experienced a relapse. Univariate analysis indicated differences in sex, modified Rankin Scale scores at onset, movement disorders, central hypoventilation, and intensive care unit occupancy between the good- and poor- outcome groups. Patients with anti-LGI1 and anti-CASPR2 encephalitis showed similar clinical manifestations while presenting delineating characteristics. Those with VGKC antibody-associated diseases generally responded well to immunotherapy and demonstrated favorable clinical outcomes. Several factors affected the prognosis, and a long-term follow-up examination is necessary.

Keywords: Anti-CASPR2 encephalitis; Anti-LGI1 encephalitis; Autoimmune encephalitis; Prognosis; VGKC.

MeSH terms

  • Autoantibodies
  • China / epidemiology
  • Contactin 2
  • Encephalitis* / diagnosis
  • Encephalitis* / therapy
  • Female
  • Glioma*
  • Hashimoto Disease
  • Humans
  • Intracellular Signaling Peptides and Proteins
  • Leucine
  • Male
  • Potassium Channels, Voltage-Gated*
  • Retrospective Studies

Substances

  • Autoantibodies
  • Contactin 2
  • Intracellular Signaling Peptides and Proteins
  • Potassium Channels, Voltage-Gated
  • Leucine

Supplementary concepts

  • Hashimoto's encephalitis