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Kelch-like protein 11 antibodies in seminoma-associated paraneoplastic encephalitis

New England Journal of Medicine Jul 11, 2019

Mandel-Brehm C, et al. - Researchers reported on the case of a 37-year-old man with a history of seminoma who presented with vertigo, ataxia, and diplopia. Programmable phage display revealed the presence of an autoantibody specific for kelch-like protein 11 (KLHL11). Using immunoassays, KLHL11 IgG was identified in 12 other men with comparable neurologic features and testicular disease. They immunostained the patient’s IgG on mouse brain tissue and identified that multiple brain regions had scant but distinguishing points of staining, with enrichment in perivascular and perimeningeal tissues. In 9 of the 13 patients, the diagnosis of seminoma was preceded by the onset of the neurologic syndrome. In Olmsted County, Minnesota, an age-adjusted estimate of the prevalence of autoimmune KLHL11 encephalitis was 2.79 cases per 100,000 men.

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