KLHL11 paraneoplastic encephalitis: clinical features

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

KLHL11 paraneoplastic encephalitis: clinical features. Brainstem encephalitis, Limbic encephalitis. Fully referenced neurology checklist.

Encephalitic features

  • Brainstem encephalitis
  • Limbic encephalitis

Cerebellar features

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Cerebral features

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Myoclonic features

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Other features

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Atypical features

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References

  1. Dubey D, Wilson MR, Clarkson B, et al. Expanded clinical phenotype, oncological associations, and immunopathologic insights of paraneoplastic kelch-like protein-11 encephalitis. JAMA Neurol 2020; 77:1420-1429. 
  2. Mahmoud A, Dubey D, Goonetilleke A, Lee G, Elhabet S, Ahmad I, Dogar M, Jacob A. Paraneoplastic KLHL11 encephalitis presenting with progressive tinnitus, hearing loss and ataxia. Pract Neurol 2025; 25:443-447. 
  3. Chai S, Liu G, Zhang Y. Anti-kelch-like protein 11 antibody-associated encephalitis: two case reports and literature review. BMC Neurol 2025; 25:297.
  4. Jin M, Brand T, Erdag Turgeon E, et al. Clinical manifestations, tumor associations, and long-term outcomes of anti-KLHL11 encephalitis. Neurol Neuroimmunol Neuroinflamm 2026; 13:e200597. 
  5. Schwarz FK, Höftberger R, Endmayr V, et al. Vestibular and ocular motor phenotype in a case of paraneoplastic autoimmune kelch-like protein-11 encephalitis. Neurol Neuroimmunol Neuroinflamm 2026; 13:e200576. 
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