Paraneoplastic Neurologic syndromes (PNS) are rare heterogeneous group of disorders associated with cancer, caused by mechanisms other than, direct infiltration, metastasis, infection, coagulopathy, metabolic and nutritional deficits or consequences of therapy. These syndromes can affect any area of the nervous system, including central nervous system, peripheral and autonomic nervous system, neuromuscular junction, and muscles. Over the last five decades three waves of interest are noted in paraneoplastic neurological syndromes: First, the clinical and pathological recognition of PNS; and then the discovery of immunological mechanisms characterized by the presence of antibodies and associated cytotoxic T cell responses against intracellular antigens, and more recently, the surge of antibodies ('onconeural' antibodies) against synaptic and cellular surface proteins Although paraneoplastic neurological syndromes are uncommon and not completely understood, their antibody associations are important because of the antibody's aetiological connection with the underlying neoplasm. The paraneoplastic autoantibody may provide a roadmap to early diagnosis of the underlying neoplasm, prompt treatment of which can sometimes improve the neurological outcome. The disorder often predate the diagnosis of the underlying neoplasm, providing a pathway for detecting cancer at an earlier and amenable stage. Evaluations for paraneoplastic antibodies are sometimes negative but do not rule out the diagnosis of a paraneoplastic disorder. Therefore, relevant clinical presentations should suggest a paraneoplastic syndrome, even in the absence of paraneoplastic antibodies and prompt search for occult neoplasm. |