MR SPECTROSCOPY & VOXEL-BASED MORPHOMETRY OF THE CEREBELLUM IN LONG-TERM

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得主要资金， 因此可以在其他CRISP条目中表示。所列机构为 中心，不一定是研究者的机构。 前言：眼阵挛共济失调（OA）是一种副肿瘤，可能是自身免疫性表现的隐匿性神经母细胞瘤在幼儿。典型的发病年龄是1-3岁，典型的肿瘤是一个小的，有些分化的，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系统状况。治疗包括免疫抑制药物，这往往是必要的几年。结果并不理想：大多数患有OA的儿童都有严重的发育和行为问题。尽管存在运动、认知和行为后遗症，但患有副肿瘤性OA的儿童的典型神经影像学表现正常。 我们建议使用新的神经影像学方法来寻找小脑组织块或神经元丢失的细微损失 具体目标1：使用一种新的图像处理技术，基于体素的形态测量，特别强调小脑体积，将患有OA的儿童与正常儿童进行比较。 具体目标二：使用磁共振波谱分析比较患有OA的儿童与正常儿童，重点是小脑，以评估无“萎缩”的轻微神经元丢失。将来自小脑的MRS与其他脑区域以及正常比较受试者进行比较。

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. Introduction: 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 developmental and behavioral problems. Despite ongoing motor, cognitive and behavioral sequelae, typical neuroimaging of children who have had paraneoplastic OA is normal. We propose use of newer neuroimaging modalities to look for subtle loss of tissue bulk or neuronal loss in the cerebellum Specific Aim 1: To compare children who have had OA with normal children using a new image processing technique, voxel based morphometry, with particular emphasis on cerebellar volume. Specific Aim 2: To compare children who have had OA with normal children using MR spectroscopy, with emphasis on the cerebellum, to assess for subtle neuronal loss without "atrophy". MRS from cerebellum will be compared to other brain regions as well as to normal comparison subjects.