77 - Paraneoplastic neurological antibodies: Nuclear

Abstract

Paraneoplastic anti-neuronal antibodies bind to antigens in Purkinje cells and other neurones. The antigens are generally neuronal specific and their cellular location is both cytoplasmic and nuclear. The antibodies are found in the serum of a significant proportion of patients with paraneoplastic neurological syndromes. These rare debilitating disorders manifest themselves as defects in coordination. Although the anti-neuronal antibodies are rare, they do have an association with particular tumours, most commonly small cell carcinomas of the lung, breast tumors and ovarian tumors. They are also found in patients with neurological syndromes of unknown aetiology and occasionallyin healthy individuals. Their presence should, therefore, not be taken as the only condition for defining the neurological syndrome as paraneoplastic. However, the association with paraneoplastic neurological syndromes is such that their identification warrants a thorough examination for an associated tumor. Indirect immunofluorescence on cerebellar tissue is the initial screen for anti-neuronal antibodies and Western blots with cerebellar extracts are used to confirm the specificities. This chapter is concerned with paraneoplastic neurological antibodies that target neuronal nuclei of the central nervous system, namely anti-Hu (ANNA-1), anti-Ri (ANNA-2), ANNA-3 and anti-Ma (Ma1, Ma2/Ta, and Ma3).