Program in Neuroscience and Mental Health, Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada.
Neuro Oncol. 2012 Oct;14(10):1294-303. doi: 10.1093/neuonc/nos160. Epub 2012 Sep 5.
Cerebellar mutism syndrome (CMS) is an important medical challenge in the management of pediatric posterior fossa brain tumors, because it occurs in a subset of children following tumor resection. A definitive clinical profile and neuroanatomical substrate associated with CMS remains unclear. We investigated the relationship between presurgical and clinical variables and the incidence of CMS, along with diffusion tensor imaging, to characterize the integrity of cerebello-thalamo-cerebral white matter pathways. Seventeen children with posterior fossa tumors and CMS, 34 children with posterior fossa tumors without CMS, and 28 healthy children were enrolled in this study. Bilateral cerebello-thalamo-cerebral pathways were delineated and segmented into anatomical regions. Mean integrity measures for each region were compared among children with CMS, children without CMS, and healthy children. Left-handedness, medulloblastoma histology, and larger tumor size distinguished between patients with CMS and patients without CMS (P < .04). Right cerebellar white matter within the cerebello-thalamo-cerebral pathway was compromised in children with CMS relative to children without CMS and healthy children (P < .02). We provide a potential schema for CMS risk among children treated for posterior fossa tumors. Left-handed children treated for medulloblastoma may be the most at risk for CMS, and unilateral, localized damage within the cerebello-thalamo-cerebral pathway at the level of the right cerebellum is implicated in the presentation of CMS. This disruption in communication between the right cerebellum and left frontal cortex may contribute to speech-language problems observed in children with CMS. Our findings may be relevant for surgical planning and speech-language therapy to mitigate symptoms of CMS.
小脑缄默症(CMS)是儿童后颅窝脑肿瘤管理中的一个重要医学挑战,因为它发生在肿瘤切除后一部分儿童中。与 CMS 相关的明确临床特征和神经解剖学基础仍不清楚。我们研究了术前和临床变量与 CMS 发生率之间的关系,以及弥散张量成像,以表征小脑-丘脑-大脑白质通路的完整性。本研究纳入了 17 例后颅窝肿瘤伴 CMS 的儿童、34 例后颅窝肿瘤无 CMS 的儿童和 28 例健康儿童。双侧小脑-丘脑-大脑通路被描绘并分割成解剖区域。对 CMS 患儿、无 CMS 患儿和健康儿童的每个区域的平均完整性测量值进行了比较。左撇子、髓母细胞瘤组织学和较大的肿瘤大小将 CMS 患儿和无 CMS 患儿区分开来(P <.04)。与无 CMS 患儿和健康儿童相比,CMS 患儿的右侧小脑白质在小脑-丘脑-大脑通路内受到损害(P <.02)。我们为接受后颅窝肿瘤治疗的儿童提供了 CMS 风险的潜在模式。接受髓母细胞瘤治疗的左撇子儿童可能面临最高的 CMS 风险,小脑-丘脑-大脑通路中单侧、局部的右小脑损伤与 CMS 的表现有关。这种小脑右侧与左侧额叶皮层之间的通讯中断可能导致 CMS 患儿出现言语语言问题。我们的发现可能与手术计划和言语语言治疗相关,以减轻 CMS 的症状。