Anti CASPR2 VGKC autoimmune encephalitis: management

Evidence-based neurology checklist on anti caspr2 vgkc autoimmune encephalitis: management: Anti VGKC antibody testing Positive VGKC antibody test is not pathogenic unless either CASPR2 or LGI1 is positive Anti-CASPR2 may co-exist with anti-LGI1: this is double seropositivity Serum neurofilament…

Anti VGKC antibody testing

  • Positive VGKC antibody test is not pathogenic unless either CASPR2 or LGI1 is positive
  • Anti-CASPR2 may co-exist with anti-LGI1: this is double seropositivity

Serum neurofilament light (NfL) testing

Initial treatment

Secondary treatment for refractory cases

References

  1. Lee R, Buckley C, Irani SR, Vincent A. Autoantibody testing in encephalopathies. Pract Neurol 2012; 12:4-13.
  2. van Sonderen A, Ariño H, Petit-Pedrol M, et al. The clinical spectrum of Caspr2 antibody-associated disease. Neurology 2016; 87:521-528. 
  3. Binks SNM, Klein CJ, Waters P, Pittock SJ, Irani SR. LGI1, CASPR2 and related antibodies: a molecular evolution of the phenotypes. JNNP 2017; pii: jnnp-2017-315720 (Epub ahead of print).
  4. Lang B, Makuch M, Moloney T, et al. Intracellular and non-neuronal targets of voltage-gated potassium channel complex antibodies. JNNP 2017; 88:353-361. 
  5. van Sonderen A, Schreurs MW, de Bruijn MA, et al. The relevance of VGKC positivity in the absence of LGI1 and Caspr2 antibodies. Neurology 2016; 86:1692-1699. 
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