{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T06:03:40Z","timestamp":1789625020678,"version":"build-2803163510"},"reference-count":27,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T00:00:00Z","timestamp":1789603200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Anti-Kelch-like protein 11 (KLHL11) antibody encephalitis, first reported in 2019, is a rare autoimmune disorder typically associated with seminoma. This disorder exhibits a striking male predominance, with cerebellar ataxia as the hallmark manifestation, whereas seizures and altered consciousness occur less frequently. Cases without detectable malignancy account for fewer than 20% of reports, and their clinical manifestations, treatment responses, and outcomes have not been systematically characterized.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We report a case of a 62-year-old male with anti-KLHL11 encephalitis and no detectable malignancy, who initially presented with recurrent generalized tonic-clonic seizures (GTCS), followed by intermittent seizures and progressive neurological deterioration. A systematic search of published cases with narrative synthesis was conducted to analyze the clinical presentation, neuroimaging features, treatment responses, and outcomes of similar reported cases.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      We analyzed 13 cases of anti-KLHL11 encephalitis without detectable malignancy. The most common clinical manifestations were ataxia (9\/13), dysarthria (8\/13), and gaze palsy\/nystagmus (6\/13). Intracranial pressure (ICP) (median: 165 mmH\n                      <jats:sub>2<\/jats:sub>\n                      O, range: 60\u2013200 mmH\n                      <jats:sub>2<\/jats:sub>\n                      O), cerebrospinal fluid (CSF) leukocyte count (median: 9 \u00d7 10\n                      <jats:sup>6<\/jats:sup>\n                      \/L, range: 1\u201346 \u00d7 10\n                      <jats:sup>6<\/jats:sup>\n                      \/L), and CSF protein level (median: 0.50 g\/L, range: 0.27\u20131.32 g\/L) were also measured. The median serum anti-KLHL11 antibody titer was 1:100 (range: 1:30\u20131:160,000), while CSF anti-KLHL11 antibody titer was 1:10 (range: 1:1\u20131:32). Electroencephalography (EEG) was normal in 2 cases, showed epileptiform discharges in 2, diffuse background slowing in 2, and sleep-related central hypoventilation in 1. Brain magnetic resonance imaging (MRI) was unremarkable in 3 cases. Seven cases (53.8%) presented with T2\/fluid-attenuated inversion recovery (FLAIR) hyperintensities, 4 cases (30.8%) showed cerebellar\/brainstem atrophy, 1 case (7.7%) presented with acute cerebral infarction. Following immunotherapy, 6 patients achieved clinical improvement, 3 patients remained clinically stable, 4 deteriorated clinically, of whom 2 died of central respiratory failure.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Anti-KLHL11 encephalitis without detectable malignancy cannot be reliably distinguished from tumor-positive cases based solely on clinical symptoms and neuroimaging findings. The gold standard for differential diagnosis remains comprehensive tumor screening and long-term follow-up. Extensive white matter and deep gray matter involvement, together with prominent seizures, may be more prevalent in patients without detectable malignancy, although these observations require validation in larger comparative cohorts.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fimmu.2026.1913121","type":"journal-article","created":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T05:31:38Z","timestamp":1789623098000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Anti-KLHL11 antibody encephalitis without detectable malignancy: a case report and literature review"],"prefix":"10.3389","volume":"17","author":[{"given":"Chenxin","family":"Yao","sequence":"first","affiliation":[{"name":"Department of Neurology, the First Hospital of Jilin University, Jilin University","place":["Jilin, Changchun, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mengtan","family":"Cai","sequence":"additional","affiliation":[{"name":"Department of Neurology, the First Hospital of Jilin University, Jilin University","place":["Jilin, Changchun, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zixuan","family":"Shen","sequence":"additional","affiliation":[{"name":"Department of Neurology, the First Hospital of Jilin University, Jilin University","place":["Jilin, Changchun, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoxu","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Neurology, the First Hospital of Jilin University, Jilin University","place":["Jilin, Changchun, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jie","family":"Cao","sequence":"additional","affiliation":[{"name":"Department of Neurology, the First Hospital of Jilin University, Jilin 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