Anti LGI1 VGKC autoimmune encephalitis: clinical features

Evidence-based neurology checklist on anti lgi1 vgkc autoimmune encephalitis: clinical features: Epidemiology Anti LG1 accounts for most cases of anti VGKC encephalitis It may co-exist with anti CASPR2 antibodies (double positive) Seropositivity may be delayed Onset age is about 65 years Males are…

Epidemiology

  • Anti LG1 accounts for most cases of anti VGKC encephalitis
  • It may co-exist with anti CASPR2 antibodies (double positive)
  • Seropositivity may be delayed
  • Onset age is about 65 years
  • Males are affected more frequently by a ratio of 2:1

Possible associated HLA haplotypes

Clinical triad

Psychiatric features

Cognitive features

Seizures

Other features

Outcome

Differential diagnosis

References

  1. Lee R, Buckley C, Irani SR, Vincent A. Autoantibody testing in encephalopathies. Pract Neurol 2012; 12:4-13.
  2. Vincent A, Bien CG, Irani SR, Waters P. Autoantibodies associated with diseases of the CNS: new developments and future challenges. Lancet Neurol 2011; 10:759-772.
  3. Binks SNM, Klein CJ, Waters P, Pittock SJ, Irani SR. LGI1, CASPR2 and related antibodies: a molecular evolution of the phenotypes. JNNP 2018; 89:526-534.
  4. Kim TJ, Lee ST, Moon J, et al. Anti-LGI1 encephalitis is associated with unique HLA subtypes. Ann Neurol 2017; 81:183-192. 
  5. van Sonderen A, Roelen DL, Stoop JA, et al. Anti-LGI1 encephalitis is strongly associated with HLA-DR7 and HLA-DRB4. Ann Neurol 2017; 81:193-198. 
  6. And 14 more. Subscribe to see the full list

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