Anti CASPR2 VGKC autoimmune encephalitis: clinical features
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Anti CASPR2 VGKC autoimmune encephalitis: clinical features. Hashimoto thyroiditis, Thymoma. Fully referenced neurology checklist.
Causes of anti CASPR2 positivity
- Hashimoto thyroiditis
- Thymoma
- Systemic lupus erythematosus (SLE)
- Pembrolizumab
- Association with HLA-DRB1*11:01 haplotype
Clinical presentations
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Central features
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Movement disorders
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Peripheral features
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Clinical outcome
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References
- Lee R, Buckley C, Irani SR, Vincent A. Autoantibody testing in encephalopathies. Pract Neurol 2012; 12:4-13.
- van Sonderen A, Ariño H, Petit-Pedrol M, et al. The clinical spectrum of Caspr2 antibody-associated disease. Neurology 2016; 87:521-528.
- 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.
- 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.
- 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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