TY - JOUR
T1 - Pearls & Oy-sters
T2 - KLHL11 IgG Paraneoplastic-Associated Hearing Loss and Rhombencephalitis in a Woman With Metastatic Müllerian Tumor
AU - Agyei, Paunel
AU - Chen, Michelle
AU - Guo, Yong
AU - Pérez, Carlos A.
AU - Dubey, Divyanshu
AU - Gupta, Rajesh K.
PY - 2024/4/9
Y1 - 2024/4/9
N2 - Kelch-like protein-11 (KLHL11) immunoglobulin G (IgG) is a recently reported paraneoplastic autoantibody associated with rhombencephalitis, which commonly presents with ataxia, diplopia, vertigo, hearing loss, tinnitus, and gaze palsies. The association of this high-risk paraneoplastic autoantibody with testicular germ cell tumors is widely accepted, but it has not been associated with Müllerian tumors. In this study, we report a woman without a known germ cell tumor presenting with signs and symptoms suggesting autoimmune encephalitis. She was found to have metastatic ovarian serous carcinoma with KLHL11 immunoreactivity on histopathology. This case demonstrates a rare cancer association of KLHL11 IgG-seropositive rhombencephalitis with Müllerian tumor and highlights that this autoantibody can also be detected in female patients. Thus, this case expands on the current knowledge of KLHL11-related autoimmune encephalitis including the paraneoplastic presentation, associated tumor types, and management of this syndrome in women.
AB - Kelch-like protein-11 (KLHL11) immunoglobulin G (IgG) is a recently reported paraneoplastic autoantibody associated with rhombencephalitis, which commonly presents with ataxia, diplopia, vertigo, hearing loss, tinnitus, and gaze palsies. The association of this high-risk paraneoplastic autoantibody with testicular germ cell tumors is widely accepted, but it has not been associated with Müllerian tumors. In this study, we report a woman without a known germ cell tumor presenting with signs and symptoms suggesting autoimmune encephalitis. She was found to have metastatic ovarian serous carcinoma with KLHL11 immunoreactivity on histopathology. This case demonstrates a rare cancer association of KLHL11 IgG-seropositive rhombencephalitis with Müllerian tumor and highlights that this autoantibody can also be detected in female patients. Thus, this case expands on the current knowledge of KLHL11-related autoimmune encephalitis including the paraneoplastic presentation, associated tumor types, and management of this syndrome in women.
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UR - http://www.scopus.com/inward/citedby.url?scp=85187848091&partnerID=8YFLogxK
U2 - 10.1212/WNL.0000000000209187
DO - 10.1212/WNL.0000000000209187
M3 - Article
C2 - 38484225
AN - SCOPUS:85187848091
SN - 0028-3878
VL - 102
SP - e209187
JO - Neurology
JF - Neurology
IS - 7
ER -