Another autoantibody in paraneoplastic neurological disease.

Abstract

Paraneoplastic neurological syndromes (PNS) are an uncommon group of disorders caused by immune responses primarily directed against an underlying neoplasm that cross-react with proteins expressed in the peripheral or central nervous system. Understanding the relationship between expression of specific proteins in the tumour and the autoimmune neurological disease with which they associate is an ongoing challenge. PNS are frequently associated with the presence of specific serum autoantibodies, which may be used as a diagnostic aid. For example, anti-Hu (also called anti-neuronal nuclear antibody 1, ANNA-1) is an antibody detected in the sera of patients with paraneoplastic encephalomyelitis and/or subacute sensory neuronopathy. More than 80% of anti-Hu+ patients will have an underlying tumour, most commonly a small cell lung carcinoma. Similarly, a distinctive cytoplasmic staining pattern of the Purkinje cells has been noted with sera derived from patients with paraneoplastic cerebellar degeneration and an associated gynaecological (breast or ovarian) tumour. This antibody, anti-Yo or anti-Purkinje cell antibody 1 (APCA-1), has been reported in 40% of patients with paraneoplastic cerebellar degeneration. Several other neurological syndromes have also been associated with an underlying tumour. Opsoclonus/myoclonus syndrome presents with involuntary saccadic eye movements …