• Caleigh Mandel-Brehm, Divyanshu Dubey, Thomas J Kryzer, Brian D O'Donovan, Baouyen Tran, Sara E Vazquez, Hannah A Sample, Kelsey C Zorn, Lillian M Khan, Ian O Bledsoe, Andrew McKeon, Samuel J Pleasure, Vanda A Lennon, Joseph L DeRisi, Michael R Wilson, and Sean J Pittock.
• From the Department of Biochemistry and Biophysics (C.M.-B., B.D.O., S.E.V., H.A.S., K.C.Z., L.M.K., J.L.D.), the Weill Institute for Neurosciences (B.T., I.O.B., S.J. Pleasure, M.R.W.), the Department of Neurology (B.T., I.O.B., S.J. Pleasure, M.R.W.), and the Chan Zuckerberg Biohub (J.L.D.), University of California, San Francisco, San Francisco; and the Departments of Laboratory Medicine and Pathology (D.D., T.J.K., A.M., V.A.L., S.J. Pittock), Neurology (D.D., A.M., V.A.L., S.J. Pittock), and Immunology (V.A.L.), Mayo Clinic, Rochester, MN.
• N. Engl. J. Med. 2019 Jul 4; 381 (1): 47-54.

AbstractA 37-year-old man with a history of seminoma presented with vertigo, ataxia, and diplopia. An autoantibody specific for kelch-like protein 11 (KLHL11) was identified with the use of programmable phage display. Immunoassays were used to identify KLHL11 IgG in 12 other men with similar neurologic features and testicular disease. Immunostaining of the patient's IgG on mouse brain tissue showed sparse but distinctive points of staining in multiple brain regions, with enrichment in perivascular and perimeningeal tissues. The onset of the neurologic syndrome preceded the diagnosis of seminoma in 9 of the 13 patients. An age-adjusted estimate of the prevalence of autoimmune KLHL11 encephalitis in Olmsted County, Minnesota, was 2.79 cases per 100,000 men. (Funded by the Rochester Epidemiology Project and others.).Copyright © 2019 Massachusetts Medical Society.

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