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Letter
Purkinje cell cytoplasmic antibody type I (anti-Yo): predictive of gastrointestinal adenocarcinomas in men
  1. Jenny Linnoila1,
  2. Yong Guo2,
  3. Avi Gadoth1,2,
  4. Aditya Raghunathan2,
  5. Becky Parks3,
  6. Andrew McKeon1,2,
  7. Claudia F Lucchinetti1,
  8. Vanda A Lennon1,2,4,
  9. Sean J Pittock1,2
  1. 1Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
  2. 2Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA
  3. 3Department of Neurology, Washington University, St Louis, Missouri, USA
  4. 4Department of Immunology, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr Sean J Pittock, Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA; pittock.sean{at}mayo.edu

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Introduction

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.

Methods

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

[SP1.pdf]

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).

Figure 1

PCA-1 autoantigen expression in tumour of patient #1: (A) Oesophageal mucosa invaded by poorly differentiated adenocarcinoma (H&E stain). (B) On immunohistochemical staining, CDX2 positivity is demonstrated, supporting origin from the gastrointestinal tract. (C) Human cerebellar Purkinje neurons stain positively for CDR2. (D) Malignant cells are identified in the original tumour biopsy by their abnormally large size and elongated nuclei with increased nuclear–cytoplasmic ratio. CDR2 immunoreactivity is demonstrated in the cytoplasm of patient #1’s malignant cells (arrow). (E) Malignant cells …

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