Pitsika Marina, Tsitouras Vassilios
Department of Pediatric Neurosurgery, Mitera Children's Hospital, Athens, Greece.
J Neurosurg Pediatr. 2013 Dec;12(6):604-14. doi: 10.3171/2013.8.PEDS13168. Epub 2013 Sep 27.
Mutism of cerebellar origin is a well-described clinical entity that complicates operations for posterior fossa tumors, especially in children. This review focuses on the current understanding of principal pathophysiological aspects and risk factors, epidemiology, clinical characteristics, treatment strategies, and outcome considerations. The PubMed database was searched using the term cerebellar mutism and relevant definitions to identify publications in the English-language literature. Pertinent publications were selected from the reference lists of the previously identified articles. Over the last few years an increasing number of prospective studies and reviews have provided valuable information regarding the cerebellar mutism syndrome. Importantly, the clarification of principal terminology that surrounds the wide clinical spectrum of the syndrome results in more focused research and more effective identification of this entity. In children who undergo surgery for medulloblastoma the incidence of cerebellar mutism syndrome was reported to be 24%, and significant risk factors so far are brainstem involvement and midline location of the tumor. The dentate-thalamo-cortical tracts and lesions that affect their integrity are considered significant pathophysiological issues, especially the tract that originates in the right cerebellar hemisphere. Moderate and severe forms of the cerebellar mutism syndrome are the most frequent types during the initial presentation, and the overall neurocognitive outcome is not as favorable as thought in the earlier publications. Advanced neuroimaging techniques could contribute to identification of high-risk patients preoperatively and allow for more effective surgical planning that should focus on maximal tumor resection with minimal risk to important neural structures. Properly designed multicenter trials are needed to provide stronger evidence regarding effective prevention of cerebellar mutism and the best therapeutic approaches for such patients with a combination of pharmacological agents and multidisciplinary speech and behavior augmentation.
小脑源性缄默症是一种已被充分描述的临床病症，它会使后颅窝肿瘤手术复杂化，尤其是在儿童中。本综述聚焦于对其主要病理生理方面、危险因素、流行病学、临床特征、治疗策略及预后考量的当前认识。使用“小脑缄默症”一词及相关定义在PubMed数据库中进行检索，以识别英文文献中的出版物。从先前识别文章的参考文献列表中挑选相关出版物。在过去几年中，越来越多的前瞻性研究和综述提供了有关小脑缄默症综合征的宝贵信息。重要的是，围绕该综合征广泛临床谱的主要术语的明确，导致了更有针对性的研究以及对该病症更有效的识别。据报道，在接受髓母细胞瘤手术的儿童中，小脑缄默症综合征的发生率为24%，迄今为止重要的危险因素是脑干受累和肿瘤的中线位置。齿状核 - 丘脑 - 皮质束及其完整性受影响的病变被认为是重要的病理生理问题，尤其是起源于右侧小脑半球的束。中度和重度形式的小脑缄默症综合征是初始表现中最常见的类型，总体神经认知预后并不像早期出版物中认为的那样良好。先进的神经影像学技术有助于术前识别高危患者，并允许进行更有效的手术规划，该规划应侧重于在对重要神经结构风险最小的情况下进行最大程度的肿瘤切除。需要设计合理的多中心试验，以提供更有力的证据，证明有效预防小脑缄默症以及针对此类患者联合使用药物和多学科言语及行为强化的最佳治疗方法。
