Neurocognitive outcome 12 months following cerebellar mutism syndrome in pediatric patients with medulloblastoma
Neurocognitive outcome 12 months following cerebellar mutism syndrome in pediatric patients with medulloblastoma
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DOI：
10.1093/neuonc/noq094
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发表时间：
2010-12-01
期刊：
影响因子：
15.9
通讯作者：
Gajjar, Amar
中科院分区：
文献类型：
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作者：
Palmer, Shawna L.;Hassall, Tim;Gajjar, Amar
目的是前瞻性地评估在后颅窝胚胎性肿瘤手术切除后出现小脑性缄默综合征（CMS）的儿童的早期神经认知结果，并与精心匹配的对照患者进行比较。参与正在进行的经机构审查委员会批准的胚胎性肿瘤治疗方案、被诊断为术后CMS且在诊断后12个月完成前瞻性计划的神经心理学评估的儿童被视为符合条件。将这些患者的认知结果与来自同一治疗方案且在主要诊断、诊断年龄和风险/相应治疗方面匹配的无CMS患者进行比较（n = 22对）。其中17对还根据性别进行了匹配，14对还根据种族进行了匹配。高危患者接受36 - 39.6 Gy的全脑全脊髓照射（CSI）以及对原发部位进行3D适形推量至55.8 - 59.4 Gy。中危患者接受23.4 Gy的CSI以及对原发部位进行3D适形推量至55.8 Gy。在多项认知结果上发现了显著的组间差异。匹配的对照患者表现处于平均水平，而术后出现CMS的患者在处理速度、注意力、工作记忆、执行过程、认知效率、阅读、拼写和数学方面的表现显著较差。接受髓母细胞瘤治疗且出现术后CMS的患者神经认知障碍的风险增加，早在诊断后12个月就很明显。这项研究强调了进行神经心理学评估仔细随访以及为患者及其家属提供关键支持的必要性。
The aim is to prospectively assess early neurocognitive outcome of children who developed cerebellar mutism syndrome (CMS) following surgical resection of a posterior fossa embryonal tumor, compared with carefully matched control patients. Children who were enrolled on an ongoing IRB-approved protocol for treatment of embryonal tumors, were diagnosed with postoperative CMS, and had completed prospectively planned neuropsychological evaluation at 12 months postdiagnosis were considered eligible. The cognitive outcomes of these patients were examined in comparison to patients without CMS from the same treatment protocol and matched with regard to primary diagnosis, age at diagnosis, and risk/corresponding treatment (n = 22 pairs). Seventeen were also matched according to gender, and 14 were also matched according to race. High-risk patients received 36-39.6 Gy CSI and 3D conformal boost to the primary site to 55.8-59.4 Gy. Average-risk patients received 23.4 Gy CSI and 3D conformal boost to the primary site to 55.8 Gy. Significant group differences were found on multiple cognitive outcomes. While the matched control patients exhibited performance in the average range, patients who developed CMS postsurgery were found to have significantly lower performance in processing speed, attention, working memory, executive processes, cognitive efficiency, reading, spelling, and math. Patients treated for medulloblastoma who experience postoperative CMS show an increased risk for neurocognitive impairment, evident as early as 12 months following diagnosis. This study highlights the need for careful follow-up with neuropsychological evaluation and for obtaining critical support for patients and their families.