PILOT STUDY: OPSOCLONUS ATAXIA (OA)

这个子项目是利用由NIH/NCRR资助的中心拨款提供的资源的许多研究子项目之一。子项目和调查员(PI)可能从另一个NIH来源获得了主要资金，因此可能会出现在其他CRISE条目中。列出的机构是针对中心的，而不一定是针对调查员的机构。眼阵挛共济失调是一种儿童隐匿性神经母细胞瘤的副肿瘤性自身免疫性表现。典型的发病年龄是1-3岁，典型的肿瘤是一种小的、稍有分化的I或II期神经母细胞瘤，没有n-myc扩增。肿瘤切除不会改变神经状况。治疗包括免疫抑制药物，这通常是几年所必需的。结果并不是最理想的：大多数患有骨性关节炎的儿童都有严重的发育和行为问题。尽管有持续的运动、认知和行为后遗症，但患有副肿瘤性骨性关节炎的儿童的典型神经成像是正常的。具体目的：重新检查以前接受过评估的患有骨性关节炎的儿童，以确定认知、行为和运动障碍是随着受试者年龄的增长而进展的，还是静态的。

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.