Paraneoplastic Syndrome

J Korean Neurol Assoc > Volume 20(3); 2002 > Article

Journal of the Korean Neurological Association 2002;20(3): 215-222.

방종양성증후군(傍腫瘍成症候群)

김광국 ,

울산대학교 의과대학 서울아산병원 신경과학교실

Paraneoplastic Syndrome

Kwang-kuk Kim

"Department of Neurology, University of Ulsan College of Medicine. Asan Medical Center, Seoul, Korea"

Abstract

"Paraneoplastic syndrome is a rare neurologic disorder caused not by the direct invasion or metastasis of cancer, but by the remote effects of cancer. The central- and peripheral- nervous system or neuromuscular junction area were involved in this syndrome. The pathogenesis was thought as the autoimmune disease, the result of an immunologic response to cancer and to cross-react with self-cells of the nervous system or of the neuromuscular junction, causing neuronal dam-age. Specific forms of this syndrome are often associated with specific paraneoplastic autoantibodies and cancer. The onset of neurological symptoms and detection of these antibodies often precede the diagnosis of the cancer; therefore, detection of these antibodies greatly assists the diagnosis of this syndrome and prompts investigations for the underly-ing cancer. Some paraneoplastic central neurological syndromes, such as cerebellar degeneration, limbic encephalitis, and necrotizing myelitis, were not improved by putative pathogenic autoantibodies, or by immunosuppresant or tumor removal, inspite of improvement in other peripheral neurologic syndrome, Lambert-Eaton myasthenic syndrome, neu-romyotonia, and Stiff-man syndrome. A more detailed understanding of the relationship between the cancer and the neural involvement from the molecular biological standpoint may lead to rational tumor therapy and elucidation of the mechanism of neural death. Here, major clinical forms with well-known antineuronal antibodies and specific cancers are reviewed.Key Words : Paraneoplastic syndrome, Onconeuronal antigen, Small cell lung cancer, Anti-Hu, Anti-Ri, PCD"