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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).
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