Neurologic paraneoplastic syndromes.
Several neurologic paraneoplastic disorders are believed to be caused by an autoimmune reaction against antigen(s) co-expressed by tumour cells and neurons. Of the paraneoplastic syndromes, the evidence for an autoimmune etiology is strongest for the Lambert-Eaton myasthenic syndrome, in which autoantibodies downregulate voltage-gated calcium channels at the presynaptic nerve terminal. For other syndromes, including cerebellar degeneration, multifocal encephalomyelitis, sensory neuronopathy, limbic encephalitis, opsoclonus-myoclonus, stiff person syndrome, and retinal degeneration, the autoimmune theory is supported by the presence of specific antineuronal antibodies. These antibodies serve as a useful diagnostic tool, but their actual role in causing neuronal injury and clinical disease remains unclear. Further understanding of immunopathogenesis awaits successful experimental models. Among different syndromes, a varied proportion of patients shows neurologic improvement with immunosuppressive treatments; it is likely that many patients have already suffered irreversible neuronal injury at the time of diagnosis.
Dropcho
E J
EJ
Department of Neurology, Indiana University Medical Center, Indianapolis 46202, USA. dropcho@iucc.iupui.edu
D000818:Animals; D001327:Autoimmune Diseases / Q000473:pathology / Q000503:physiopathology / Q000523:psychology; D006801:Humans; D009423:Nervous System Neoplasms / Q000473:pathology / Q000503:physiopathology* / Q000523:psychology; D010257:Paraneoplastic Syndromes / Q000473:pathology / Q000503:physiopathology* / Q000523:psychology