Neuroimmunology of OMS and ANNA-1/anti-Hu paraneoplastic syndromes in a child with neuroblastoma
نویسندگان
چکیده
Paraneoplastic neurologic disorders have been at the frontier of neuroimmunology for decades and remain urgent. Newer tools for the diagnosis and treatment of neuroinflammation include autoantibody screening, lymphocyte subset analysis, oligoclonal bands, and profiling of chemokines/cytokines and brainrelated proteins in CSF and blood/serum. Opsoclonus-myoclonus syndrome (OMS), a paraneoplastic disorder without a known diagnostic antibody marker, is associated with remote neuroblastoma and is often relapsing. Paraneoplastic encephalomyelitis with type 1 antineuronal nuclear antibodies (ANNA1) (alias anti-Hu) also may occur in children with neuroblastoma and exhibits intrathecal Band Tcell inflammation in adults. We now provide a detailed neuroimmunologic profile of a child with both relapsing OMS and ANNA-1 paraneoplastic syndromes and neuroblastoma.
منابع مشابه
Opsoclonus-myoclonus and anti-Hu positive limbic encephalitis in a patient with neuroblastoma.
Opsoclonus-myoclonus syndrome (OMS) is seen in 2-3% of children with neuroblastoma and is believed to be caused by an autoimmune process elicited by the tumor. Although long-term neurologic sequelae are common in children with OMS, limbic encephalitis has not previously been reported. We report a child who developed limbic encephalitis associated with anti-Hu antibodies, 6 years after her initi...
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Opsoclonus-myoclonus syndrome (OMS) may be associated with ANNA-1 (anti-Hu) autoantibodies. The standard treatment with IVIG, steroids, and anti-CD20 monoclonal antibody may fail, and optimal therapy is unknown. A patient developed OMS with high-titer ANNA-1 following recovery from neuroblastoma. She failed standard therapy and had only transient response to rituximab. Treatment with the humani...
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