Anti LGI1 VGKC autoimmune encephalitis: clinical features
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Anti LGI1 VGKC autoimmune encephalitis: clinical features. Anti LG1 accounts for most cases of anti VGKC encephalitis, Seropositivity may be delayed.
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
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Clinical triad
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Psychiatric features
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Cognitive features
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Seizures
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Other features
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Outcome
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Differential diagnosis
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References
- Lee R, Buckley C, Irani SR, Vincent A. Autoantibody testing in encephalopathies. Pract Neurol 2012; 12:4-13.
- 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.
- 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.
- Kim TJ, Lee ST, Moon J, et al. Anti-LGI1 encephalitis is associated with unique HLA subtypes. Ann Neurol 2017; 81:183-192.
- 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.
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