Abstract
A 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.
Original language | English (US) |
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Pages (from-to) | 47-54 |
Number of pages | 8 |
Journal | New England Journal of Medicine |
Volume | 381 |
Issue number | 1 |
DOIs | |
State | Published - Jul 4 2019 |
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ASJC Scopus subject areas
- Medicine(all)
Cite this
Kelch-like Protein 11 antibodies in seminoma-Associated paraneoplastic encephalitis. / Mandel-brehm, Caleigh; Dubey, Divyanshu; Kryzer, Thomas J.; O'donovan, Brian D.; Tran, Baouyen; Vazquez, Sara E.; Sample, Hannah A.; Zorn, Kelsey C.; Khan, Lillian M.; Bledsoe, Ian O.; Mckeo, Andrew; Pleasure, Samuel J.; Lennon, Vanda A; Derisi, Joseph L.; Wilson, Michael R.; Pittock, Sean J.
In: New England Journal of Medicine, Vol. 381, No. 1, 04.07.2019, p. 47-54.Research output: Contribution to journal › Article
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TY - JOUR
T1 - Kelch-like Protein 11 antibodies in seminoma-Associated paraneoplastic encephalitis
AU - Mandel-brehm, Caleigh
AU - Dubey, Divyanshu
AU - Kryzer, Thomas J.
AU - O'donovan, Brian D.
AU - Tran, Baouyen
AU - Vazquez, Sara E.
AU - Sample, Hannah A.
AU - Zorn, Kelsey C.
AU - Khan, Lillian M.
AU - Bledsoe, Ian O.
AU - Mckeo, Andrew
AU - Pleasure, Samuel J.
AU - Lennon, Vanda A
AU - Derisi, Joseph L.
AU - Wilson, Michael R.
AU - Pittock, Sean J
PY - 2019/7/4
Y1 - 2019/7/4
N2 - A 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.
AB - A 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.
UR - http://www.scopus.com/inward/record.url?scp=85068554115&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85068554115&partnerID=8YFLogxK
U2 - 10.1056/NEJMoa1816721
DO - 10.1056/NEJMoa1816721
M3 - Article
C2 - 31269365
AN - SCOPUS:85068554115
VL - 381
SP - 47
EP - 54
JO - New England Journal of Medicine
JF - New England Journal of Medicine
SN - 1533-4406
IS - 1
ER -