Paraneoplastic syndromes affecting the nervous system.
The paraneoplastic neurologic disorders (PND) are a diverse group of diseases characterized by the presence of neurologic dysfunction in the setting of a remote cancer. PND can affect almost any part of the nervous system, and are most commonly associated with lung cancer (small cell) and gynecologic tumors. Laboratory studies have demonstrated that an autoimmune response links the neurologic disorder and the cancer, and established a model whereby the cancer is believed to initiate the syndrome by expressing a protein antigen normally expressed in the nervous system, leading to anti-tumor immune response followed by autoimmune neurologic symptoms. We review the currently known PND and their pathogenesis.
Darnell
Robert B
RB
Howard Hughes Medical Institute and Laboratory of Molecular Neuro-Oncology, The Rockefeller University, New York, NY 10021, USA. darnelr@rockefeller.edu
Posner
Jerome B
JB
D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases / Q000276:immunology; D018288:Carcinoma, Small Cell / Q000150:complications; D002493:Central Nervous System Diseases / Q000209:etiology; D005260:Female; D005833:Genital Neoplasms, Female / Q000150:complications; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications; D009422:Nervous System Diseases / Q000209:etiology*; D020511:Neuromuscular Junction Diseases / Q000209:etiology; D010257:Paraneoplastic Syndromes / Q000209:etiology*; D010523:Peripheral Nervous System Diseases / Q000209:etiology
D001323:Autoantibodies