ArticleNeurology(R) neuroimmunology & neuroinflammation2024
Immune Checkpoint Inhibitor-Associated Kelch-Like Protein-11 IgG Brainstem Encephalitis.
Article in Neurology(R) neuroimmunology & neuroinflammation, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed, 12 citations in OpenAlex.
- Checkpoint blockade-related CNS adverse events are characterized by neurotoxic T helper cells with sustained neuroantigen reactivity.Cell reports. Medicine · 2026Article
- Immune checkpoint inhibitor-related kelch-like protein 11-IgG cerebellitis successfully treated with efgartigimod as rescue therapy: a case report.Frontiers in immunology · 2026Article
- Movement Disorders in Antibody-Associated Neurologic Diseases: A Nationwide Study.Neurology(R) neuroimmunology & neuroinflammation · 2026Observational
- Anti-KLHL11 antibody encephalitis without detectable malignancy: a case report and literature review.Frontiers in immunology · 2026Review
- Case Report: KLHL11 encephalitis in a female patient with dual primary malignancies of breast and lung cancer: response to FcRn inhibitor therapy.Frontiers in immunology · 2025Article
- Evaluation of warning strategies for paraneoplastic neurological syndromes associated with PD-1/PD-L1 inhibitors.Frontiers in immunology · 2025Article
- Ten Years ofNeurology(R) neuroimmunology & neuroinflammation · 2025Article
- Immune Checkpoint Inhibitor-Associated Kelch-Like Protein-11 IgG Brainstem Encephalitis.Neurology(R) neuroimmunology & neuroinflammation · 2024Article
Corrections and comments
- Erratum issued
Authors and funding
8 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesKelch-like protein-11 (KLHL11)-IgG is associated with rhombencephalitis and seminoma. It has not previously been described as a neurologic immune checkpoint inhibitor (ICI)-related adverse event (nirAE) or in association with esophageal adenocarcinoma.
methodsWe describe a 61-year-old man with metastatic esophageal adenocarcinoma treated with folinic acid, fluorouracil, oxaliplatin (FOLFOX), and nivolumab, who subsequently developed diplopia, vertigo, and progressive gait ataxia after 8 weeks of treatment.
resultsOwing to a concern for ICI-associated myasthenia gravis, nivolumab was held and he was treated with prednisone and pyridostigmine. EMG showed no neuromuscular junction dysfunction, and acetylcholine-receptor antibodies were negative. Brain MRI was unrevealing. Murine brain tissue immunofluorescence assay revealed KLHL11-IgG in both serum and CSF, confirmed by cell-based assay. Tumor histopathology demonstrated poorly differentiated, highly proliferative adenocarcinoma with increased mitotic figures and cytoplasmic KLHL11 immunoreactivity. He was initiated on 6 months of cyclophosphamide in addition to FOLFOX for post-ICI-associated KLHL11-IgG rhombencephalitis. DISCUSSION: We report KLHL11-IgG rhombencephalitis associated with poorly differentiated esophageal cancer as a novel nirAE. Tumor staining revealed KLHL11 immunoreactivity, supporting a cancer-antigen-driven ICI-associated paraneoplastic syndrome. Recognition of novel nirAEs can expedite treatment and potentially prevent progressive neurologic disability.
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