治疗髓母细胞瘤的幸存者术后出现小脑缄默综合征后的适应、行为和情感方面的结果。

IF 2.1 3区 医学 Q3 CLINICAL NEUROLOGY Journal of neurosurgery. Pediatrics Pub Date : 2024-03-29 Print Date: 2024-06-01 DOI:10.3171/2024.1.PEDS23321
Kimberly P Raghubar, Andrew M Heitzer, Fatema Malbari, Jason Gill, Roy V Sillitoe, Livia Merrill, Johanna Escalante, M Fatih Okcu, Guillermo Aldave, Avner Meoded, Stephen Kralik, Kimberly Davis, Marina Ma, Emily A H Warren, Mark D McCurdy, Howard L Weiner, William Whitehead, Michael E Scheurer, Lisa Rodriguez, Amy Daigle, Murali Chintagumpala, Lisa S Kahalley
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引用次数: 0

摘要

目的:小儿小脑缄默综合征(CMS)患者在髓母细胞瘤术后治疗期间会出现长期的神经认知功能障碍,但其对功能或适应性结果的影响尚不清楚。本研究旨在比较有 CMS 病史和无 CMS 病史的幸存者的适应、行为和情感功能:作者对 45 名幸存者(15 名有 CMS,30 名没有 CMS)的结果进行了研究。在颅骨照射后中位 2.90 年完成了全面的神经心理学评估,其中包括由家长报告的适应、行为和情感功能测量:与无 CMS 组相比,CMS 组的实际和一般适应能力明显较差。CMS组在实践、概念和一般适应能力方面的损伤率超过了一般人群的预期损伤率。尽管 CMS 组的整体智力功能、工作记忆和处理速度较低,但智商和相关认知过程与适应结果无关。在行为和情绪结果方面,没有观察到明显的群体差异或损伤率增加:结论:与未患 CMS 的幸存者相比,患 CMS 的幸存者在整体或一般适应功能方面被评为存在明显缺陷,在治疗后数年的实践技能方面尤为薄弱。这项研究的结果表明,尽管 CMS 的症状在急性期得到了恢复,但持续存在功能缺陷的风险很高,因此需要采取干预措施来降低这种风险。
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Adaptive, behavioral, and emotional outcomes following postoperative pediatric cerebellar mutism syndrome in survivors treated for medulloblastoma.

Objective: Patients who experience postoperative pediatric cerebellar mutism syndrome (CMS) during treatment for medulloblastoma have long-term deficits in neurocognitive functioning; however, the consequences on functional or adaptive outcomes are unknown. The purpose of the present study was to compare adaptive, behavioral, and emotional functioning between survivors with and those without a history of CMS.

Methods: The authors examined outcomes in 45 survivors (15 with CMS and 30 without CMS). Comprehensive neuropsychological evaluations, which included parent-report measures of adaptive, behavioral, and emotional functioning, were completed at a median of 2.90 years following craniospinal irradiation.

Results: Adaptive functioning was significantly worse in the CMS group for practical and general adaptive skills compared with the group without CMS. Rates of impairment in practical, conceptual, and general adaptive skills in the CMS group exceeded expected rates in the general population. Despite having lower overall intellectual functioning, working memory, and processing speed, IQ and related cognitive processes were uncorrelated with adaptive outcomes in the CMS group. No significant group differences or increased rates of impairment were observed for behavioral and emotional outcomes.

Conclusions: Survivors with CMS, compared with those without CMS, are rated as having significant deficits in overall or general adaptive functioning, with specific weakness in practical skills several years posttreatment. Findings from this study demonstrate the high risk for ongoing functional deficits despite acute recovery from symptoms of CMS, highlighting the need for intervention to mitigate such risk.

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来源期刊
Journal of neurosurgery. Pediatrics
Journal of neurosurgery. Pediatrics 医学-临床神经学
CiteScore
3.40
自引率
10.50%
发文量
307
审稿时长
2 months
期刊介绍: Information not localiced
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