Anti CASPR2 VGKC autoimmune encephalitis: management

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Anti CASPR2 VGKC autoimmune encephalitis: management. Positive VGKC antibody test is not pathogenic unless either CASPR2 or LGI1 is positive.

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

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Initial treatment

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Secondary treatment for refractory cases

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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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