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Neurological disorders occur in less than 0.1% of patients with tumours. The disorder is termed “paraneoplastic” when the tumour is remote from the affected organ and the symptoms are not caused by complications due to therapy, infections or associated nutritional deficits. A cellular immune response resulting in neuronal loss is considered to be responsible for the neurological disorder. Paraneoplastic neurological syndromes can be synchronous with paraneoplastic neurological antibodies of high titre in patient sera and cerebrospinal fluid; antibodies have been reported in up to 36% of patients. The presentation of antigens by tumours is thought to trigger the production of antibodies. When the antibodies are examined immunocytochemically, they reveal binding patterns that are characteristic of the respective antibodies. This chapter is concerned with paraneoplastic neurological antibodies that target antigens in the cytoplasm of the Purkinje cell, called Purkinje cytoplasmic antibodies (PCA). There are three such antibodies: anti-Yo (PCA1), PCA2 and anti-Tr (PCA-Tr). All three are characterised although only a few cases of PCA2 antibodies have been reported. Clinically these are important diagnostic markers of the paraneoplastic neurological syndrome as the clinical symptoms frequently precede the detection of the associated tumours. The antibodies are associated with different tumours, and differentiating between them allows investigation for the associated tumour to be directed to a limited number of organs. |
