Paraneoplastic Neurologic Syndromes

Abstract

Abstract Paraneoplastic neurologic syndromes (PNS) are remote effects of cancer. Immunological factors appear important in the pathogenesis of PNS since the detection of antineuronal autoantibodies. Detection of paraneoplastic antibodies is very helpful in diagnosing an unexplained neurological syndrome as paraneoplastic and in directing the search towards the underlying neoplasm. There are seven well‐characterized paraneoplastic antibodies reactive with the onconeural Hu, Yo, Ri, Ma2, CV2, amphiphysin and recoverin antigens. Recently, anti‐VGKC and anti‐NMDA receptor antibodies were identified in patients with limbic encephalitis (LE). Thirty per cent of patients with anti‐VGKC and LE have an underlying tumour, while most patients with anti‐NMDAR and LE are young women with an ovarian teratoma. Both VGKC‐ and NMDAR‐related LE respond well to immunotherapy. In contrast, the effect of immunotherapy in patients with PNS associated with antibodies against the intracellular onconeural antigens is disappointing. These patients benefit most from early detection and treatment of the underlying tumour.