Identification of autoantigens in pediatric opsoclonus-myoclonus syndrome

@article{Blaes2007IdentificationOA,
  title={Identification of autoantigens in pediatric opsoclonus-myoclonus syndrome},
  author={Franz Blaes and Verena F{\"u}hlhuber and Klaus Theo Preissner},
  journal={Expert Review of Clinical Immunology},
  year={2007},
  volume={3},
  pages={975 - 982}
}
Pediatric opsoclonus–myoclonus syndrome (OMS) is a rare disease, including eye movement disturbances, muscle jerks, ataxia and developmental disturbances as the main symptoms. In 50% of patients, OMS is associated with a neuroblastoma as a paraneoplastic neurological syndrome. Since autoantibodies have been detected in some patients and OMS patients respond to immunosuppression, an autoimmune etiology has been suspected in OMS. A variety of autoantibodies have been reported in association with… 
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TLDR
The concept of a humoral autoimmune process in the pathogenesis of OMS is supported by specific binding of autoantibodies to the surface of neuroblastoma cells and cerebellar granular neurons.
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TLDR
Overall, in the indicated types of OM, frequent and heterogeneous immunity to neuronal autoantigens without a single specific antibody marker of OM is found, and the occasional detection of antibodies to known onconeuronal antigens probably is related to cancer‐induced immunity rather than to OM.
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TLDR
The first case of SCLC-associated OMS with high titre antimitochondrial antibodies (AMA) in serum and CSF is reported, which is a rare complication of cancer characterised by chaotic, synchronous eye movements, spontaneous muscle jerks, and ataxia.
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TLDR
Recent clinical and laboratory evidence for the autoimmune theory is surveyed and how some current therapies for OMS may exert their effects through immunomodulation is discussed, providing possible new directions for immunologic research and therapy in OMS.
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