Anti CASPR2 VGKC autoimmune encephalitis: clinical features

Evidence-based neurology checklist on anti caspr2 vgkc autoimmune encephalitis: clinical features: Causes of anti CASPR2 positivity Hashimoto thyroiditis Thymoma Systemic lupus erythematosus (SLE) Pembrolizumab Association with HLA-DRB1*11:01 haplotype Clinical presentations Central features…

Causes of anti CASPR2 positivity

  • Hashimoto thyroiditis
  • Thymoma
  • Systemic lupus erythematosus (SLE)
  • Pembrolizumab
  • Association with HLA-DRB1*11:01 haplotype

Clinical presentations

Central features

Movement disorders

Peripheral features

Clinical outcome

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. Lee CH, Lin JJ, Lin KJ, et al. Caspr2 antibody limbic encephalitis is associated with Hashimoto thyroiditis and thymoma. J Neurol Sci 2014; 341:36-40. 
  4. Castro A, Romeu JC, Geraldes R, Pereira da Silva JA. Encephalopathy with upper body hypertonia and myoclonus in patient with systemic lupus erythematosus and anti-CASPR2. Lupus 2017; 26:84-87. 
  5. Brown MP, Hissaria P, Hsieh AH, Kneebone C, Vallat W. Autoimmune limbic encephalitis with anti-contactin-associated protein-like 2 antibody secondary to pembrolizumab therapy. J Neuroimmunol 2017; 305:16-18. 
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