Guidelines on the detection of paraneoplastic anti-neuronal-specific antibodies: report from the Workshop to the Fourth Meeting of the International Society of Neuro-Immunology on paraneoplastic neurological disease, held October 22-23, 1994, in Rotterdam, The Netherlands.

Abstract

Paraneoplastic syndromes of the central nervous system (PNS) consist of a variety of neurologic disorders including encephalomyelitis (limbic encephalitis, brainstem encephalitis, and acute myelitis), sensory neuronopathy, subacute cerebellar degeneration, visual paraneoplastic syndrome, stiffman syndrome, and motor neuron disease. [1-3] For the clinician, it is often difficult to arrive at a reliable diagnosis of the neurologic syndrome and to detect the underlying tumor. Neurologic signs and symptoms, although often stereotypic, are not specific. In two-thirds of cases the underlying tumor is not discovered until after the first neurologic symptoms. [4] Furthermore, the neoplasm may be small and difficult to detect. [5-7] PNS are frequently associated with serum autoantibodies reactive with neuronal antigens that are also present in the underlying tumor. These antibodies include (1) the anti-Yo antibody, also called type 1 anti-Purkinje cell antibody (PCA-1), APCA-1, or type I antibody; (2) the anti-Hu antibody, also called type 1 anti-neuronal nuclear antibody (ANNA-1) or type IIa antibody; and (3) the anti-Ri antibody, also called type 2 anti-neuronal nuclear antibody (ANNA-2) or type IIb antibody Table 1. A clinical diagnosis of PNS is supported by the finding of these specific anti-neuronal antibodies. The detection of these antibodies should also lead to a focused search for specific underlying neoplasms. [8-12] View this table: Table 1. Criteria for identification of paraneoplastic anti-neuronal antibodies A major problem in comparing results obtained by different groups of investigators has been that individual laboratories have employed different laboratory techniques for detection of these antibodies. Although identification of these antibodies has rested on immunohistochemistry and Western blot analysis, not all laboratories have used Western blot analysis to identify the molecular weight of detected antigens. Furthermore, material from a number of animal species has been used, methods of preparing substrates for Western blot are not always spelled out, and concentrations of serum and …