Immunological markers in neurological disorders

Abstract

Neurological dysfunction results from vascular, inflammatory, degenerative, neoplastic, metabolic or genetic causes. Of particular interest is a group of neurological symptoms thought to be linked to an underlying tumour, the so-called paraneoplastic syndromes. It is considered to be due to an attempt by the immune system to subjugate the growth of the tumour by triggering an antibody response against the neuronal antigens expressed by the neoplasm. The unfortunate consequence of this is an assault by the immune components on the nervous tissue, thereby rapidly precipitating a variety of neurological deficits. Every level of the nervous system is potentially vulnerable, with the disability being considered as irreversible due to the lack of regenerative capacity of the neurons. This phenomenon is rare, occurring at an approximate frequency of less than 1% of all tumours and often accompanied by the presence of specific high-titre autoantibodies in both the cerebrospinal fluid and blood. This group of antibodies are non-pathogenic markers for paraneoplastic neurological syndromes, which have expanded to almost 20 since the discovery, in 1986, of the first clinically relevant syndrome. More recently, a new generation of antineuronal antibodies against cell surface antigens, having a direct pathogenic role in causing the disease, has emerged to complement the existing repertoire. Neuronal antibodies are useful diagnostic markers of the brain disease and also, in some cases, may reveal an underlying malignancy, thus facilitating faster diagnosis and earlier treatment with consequently better prognosis.