KLHL11 paraneoplastic encephalitis: management

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

KLHL11 paraneoplastic encephalitis: management. T2 hyperintensities on T2 FLAIR, Brainstem and cerebellar atrophy. Fully referenced neurology checklist.

Magnetic resonance imaging (MRI) brain

  • T2 hyperintensities on T2 FLAIR
  • Low-signal intensity on susceptibility-weighted imaging (SWI)
  • Brainstem and cerebellar atrophy
  • Hot-cross-bun sign

Other investigations

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Treatment

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References

  1. Ishikawa H, Mandel-Brehm C, Shindo A, et al. Long-term MRI changes in a patient with Kelch-like protein 11-associated paraneoplastic neurological syndrome. Eur J Neurol 2021; 28:4261-4266.
  2. 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. 
  3. Song Y, Hu Q, Zhang Q. Anti-Kelch-like protein 11 antibody encephalitis: a case report and literature review. Front Neurol 2023; 14:1273051.
  4. 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.

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