Paraneoplastic Kelch-like protein 11 antibody-associated cerebellar and limbic encephalitis caused by metastatic “burned-out” seminoma – A scar(r)y phenomenon
Benedek Gyöngyösi, Rita Magyar-Stang, Tímea Takács, Eszter Székely, Zsolt Illés, Christine Nilsson, Tamás Györke, Péter Barsi, Daniel Juhasz, Balázs Bánky, Dániel Bereczki, Jérôme Honnorat, Bence Gunda
Abstract
INTRODUCTION: The diagnosis of paraneoplastic neurologic syndromes is challenging when the primary tumor masquerades as scar tissue (i.e. "burned-out"). METHODS: Case report. RESULTS: A 45-year-old male patient presented with progressive cerebellar symptoms and hearing loss. Initial screening for malignancy and extensive testing of paraneoplastic and autoimmune neuronal antibodies gave negative results. Repeated whole-body FDG-PET CT revealed a single paraaortic lymphadenopathy, metastasis of a regressed testicular seminoma. Anti-Kelch-like protein-11 (KLHL11) encephalitis was finally diagnosed. CONCLUSION: Our case highlights the importance of continued efforts to find an often burned-out testicular cancer in patients with a highly unique clinical presentation of KLHL11 encephalitis.