Paraneoplastic syndromes associated with anti-Hu antibodies.
Paraneoplastic syndromes are disorders associated with cancer but without a direct effect of the tumor mass or its metastases on the nervous system. Small cell carcinoma of lung associated with paraneoplastic sensory neuronopathy and/or paraneoplastic encephalomyelitis with the presence of anti-Hu antibodies has been termed "anti-Hu syndrome." Anti-Hu associated PSN-PEM is an immune disorder in which both cell-mediated and humoral mechanisms are involved. Patients are considered affected by Anti-Hu associated PSN-PEM when they develop clinical signs and symptoms of CNS dysfunction and/or sensory neuropathy not caused by metastases or other disorders, and serum or cerebrospinal fluid is positive for Hu abs. SCLC is found in more than 90% of patients with cancer and positive Hu abs. Individual patients with Hu abs associated to SCLC may suffer PSN-PEM, limbic encephalitis, brainstem encephalopathy, opsoclonus-myoclonus, paraneoplastic cerebellar degeneration or myelopathy. Hu abs have a specificity of 99% and sensitivity of 82% in detecting paraneoplastic neurological syndromes. There are two types of treatment: the first is to treat the cancer, the second is to suppress the immune reaction with the use of corticosteroids, cyclophosphamide, azathioprine, plasma exchange, intravenous immunoglobulin and immunoadsorption; however, treatment of paraneoplastic syndromes is generally unsatisfactory.
Senties-Madrid
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Department of Neurology, National Institute of Medical Sciences and Nutrition Salvador Zubiran, Mexico City, Mexico. sentiesh@yahoo.com
Vega-Boada
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D000974:Antibodies, Antinuclear / Q000032:analysis*; D000917:Antibody Formation / Q000502:physiology; D015897:Comorbidity; D005260:Female; D006681:HLA-D Antigens / Q000032:analysis / Q000276:immunology*; D006801:Humans; D007109:Immunity / Q000502:physiology; D008297:Male; D009369:Neoplasms / Q000453:epidemiology / Q000276:immunology; D015513:Oncogene Proteins / Q000032:analysis / Q000276:immunology; D010257:Paraneoplastic Syndromes / Q000453:epidemiology* / Q000276:immunology*; D012680:Sensitivity and Specificity
D000974:Antibodies, Antinuclear; D006681:HLA-D Antigens; D015513:Oncogene Proteins