Abstract: |
Purpose: To identify serologic markers in children with paraneoplastic opsoclonus-myoclonus (POM). Materials and Methods: We examined the sera of 64 children with neuroblastoma (16 with POM and 48 age-matched and stage-matched controls) by immunohistochemistry of rat brain and human cerebellum, and by Western blot analysis of protein extracts from human Purkinje cells, cortical neurons, neuroblastoma cell lines, and HuD. Results: Using immunohistochemistry, IgG reactivity against neurons was identified in 13 of 16 POM sera (81%), and 12 of 48 non-POM sera (25%; P < 0.001). IgM antineural antibodies were present in 3 of 16 POM sera (19%) and 11 of 48 (23%) non-POM sera. Except for anti-Hu antibodies detected in 10 sera (4 with POM), no other specific reactivities were identified by Western blot analysis of neuronal or of neuroblastoma protein extracts. Conclusions: We conclude that: 1) patients with neuroblastoma and POM are more likely to harbor antineuronal antibodies than patients without POM; 2) no specific serologic marker of POM was identified, but the frequent presence of antineuronal antibodies suggests that POM is immune-mediated; and 3) anti-Hu antibodies are present in some sera from patients with neuroblastoma, irrespective of the presence of POM. |
Keywords: |
immunohistochemistry; adult; child; controlled study; human tissue; child, preschool; major clinical study; nonhuman; animals; animal tissue; cerebellum; purkinje cells; neurons; blotting, western; immunoglobulin g; infant; infant, newborn; neuroblastoma; rat; western blotting; autoimmunity; rats; myoclonus; cerebral cortex; autoantibody; opsoclonus; paraneoplastic syndrome; autoantibodies; serology; antibodies, neoplasm; immunoglobulin m; paraneoplastic syndromes, nervous system; humans; human; male; female; priority journal; article; antineuronal antibodies
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