Pathogenesis of anti-Yo positive paraneoplastic cerebellar degeneration

Abstract

Paraneoplastic cerebellar degeneration (PCD) is a neurological disorder affecting the cerebellum and is believed to be immune-mediated. Anti-Yo PCD is associated with gynaecological and breast cancer tumours and the presence of these tumours is believed to invoke an immune response. The immune response includes both anti-Yo antibodies and cytotoxic T-lymphocytes that are specific for the Cdr2 antigen expressed in Purkinje cells of the cerebellum. Although both have been implicated in the pathogenic manifestation of PCD neither have been shown to be directly involved. The detection of the anti-Yo antibody greatly aids in the diagnosis of PCD and helps in uncovering the underlying neoplasm before it becomes symptomatic. Treatment of PCD has been targeted at suppressing the immune response but has been unsuccessful so far. The lack of a suitable animal model makes it difficult to study the pathogenic process directly leaving many questions unanswered about PCD pathogenesis.