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Purkinje cell cytoplasmic antibody type I (PCA-1-IgG or anti-Yo) seropositivity associates with paraneoplastic cerebellar degeneration (PCD) and is mostly restricted to female patients with müllerian or breast cancers.1–3 PCD presents with nystagmus, dysarthria and trunk and extremity ataxia. PCA-1-IgG is extremely rare in men. Here we report the frequency, clinical characteristics and oncological associations of PCA-1-IgG seropositivity in men and review the literature.
The Mayo Clinic institutional review board approved this study. In the past 30 years, Mayo Clinic’s Neuroimmunology Laboratory identified 650 PCA-1-IgG-seropositive patients3; 8 (1.2%) were men (online supplementary table 1a), with PCA-1-IgG specificity confirmed by western blot with purified human cerebellar degeneration-related protein 2 (CDR2, the PCA-1 antigen; Euroimmun, Lubeck, Germany). Clinical information was obtained for seven by case record review and physician telephone interviews. A PubMed literature search (1990–2015) identified 10 previously published PCA-1-IgG-positive male cases (online supplementary table 1b).
Supplementary file 1
Gastrointestinal adenocarcinoma tissue, from a single patient, was stained using a mouse monoclonal human CDR2-specific IgG (1:100, LifeSpan Biosciences) to detect PCA-1 antigen (figure 1).
Contributors JJL, VAL and SJP: study concept and design. JJL and SJP: drafting of the manuscript. SJP: study supervision. All authors: acquisition, analysis or interpretation of data; critical revision of the manuscript for important intellectual content.
Funding SJP has provided consultation to Alexion Pharmaceuticals, Medimmune and Chugai Pharma USA, but has received no personal fees or personal compensation for these consulting activities. All compensation for consulting activities is paid directly to Mayo Clinic. SJP has received research support from Alexion, Medimmune and Grifols. AMcK received research support from and has consulted for Euroimmun, Medimmune and Grifols, but has not received personal compensation. CFL has received funding support from Biogen, Novartis, Mallinkrodt and shares in royalties from marketing kits for detecting AQP4 autoantibody. BP has received compensation for consulting from Novartis. BP is currently employed by Biogen.
Competing interests None declared.
Ethics approval Mayo Clinic Institutional Review Board.
Provenance and peer review Not commissioned; externally peer reviewed.
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