This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Opsoclonus-ataxia (OA) is a paraneoplastic, presumably autoimmune manifestation of occult neuroblastoma in young children. Typical age at presentation is 1-3 years, and the typical tumor is a small, somewhat differentiated, stage I or II neuroblastoma, without n-MYC amplification. Tumor removal does not alter the neurological condition. Treatment consists of immunosuppressive medication, which often is necessary for several years. Outcome is not optimal: most children who have had OA have substantial development and behavioral problems. Despite ongoing motor, cognitive and behavioral sequelae, typical neuroimaging of children who have had paraneoplastic OA is normal.
SPECIFIC AIM : to reexamine children who have had OA who were previously evaluated to determine whether cognitive, behavioral and motor deficits are progressive with increasing age of subjects, or static.
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