Skye Jax, Bruss Joel, Toescu Sebastian, Aquilina Kristian, Grafft Amanda, Bardi Lola Gino, Boes Aaron D
Department of Neurology, University of Iowa Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA.
Department of Pediatrics, University of Iowa Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA.
Brain Commun. 2024 Jun 5;6(4):fcae197. doi: 10.1093/braincomms/fcae197. eCollection 2024.
Approximately 25% of paediatric patients who undergo cerebellar tumour resection develop cerebellar mutism syndrome. Our group recently showed that damage to the cerebellar deep nuclei and superior cerebellar peduncles, which we refer to as the cerebellar outflow pathway, is associated with an increased risk of cerebellar mutism syndrome. Here, we tested whether these findings replicate in an independent cohort. We evaluated the relationship between lesion location and the development of cerebellar mutism syndrome in an observational study of 56 paediatric patients ranging from five months to 14 years of age who underwent cerebellar tumour resection. We hypothesized that individuals who developed cerebellar mutism syndrome after surgery, relative to those who did not, would have lesions that preferentially intersect with: (i) the cerebellar outflow pathway and (ii) a previously generated 'lesion-symptom map' of cerebellar mutism syndrome. Analyses were conducted in accordance with pre-registered hypotheses and analytic methods (https://osf.io/r8yjv/). We found supporting evidence for both hypotheses. Compared to patients who did not develop cerebellar mutism syndrome, patients with cerebellar mutism syndrome ( = 10) had lesions with greater overlap with the cerebellar outflow pathway (Cohen's d = 0.73, = 0.05), and the cerebellar mutism syndrome lesion-symptom map (Cohen's d = 1.1, = 0.004). These results strengthen the association of lesion location with the risk of developing cerebellar mutism syndrome and demonstrate generalizability across cohorts. These findings may help to inform the optimal surgical approach to paediatric cerebellar tumours.
接受小脑肿瘤切除术的儿科患者中，约25%会发生小脑缄默综合征。我们团队最近发现，小脑深部核团和小脑上脚（我们称之为小脑传出通路）受损与小脑缄默综合征风险增加有关。在此，我们测试了这些发现能否在一个独立队列中得到重复验证。在一项观察性研究中，我们评估了56例年龄从5个月至14岁、接受小脑肿瘤切除术的儿科患者的病变位置与小脑缄默综合征发生之间的关系。我们假设，术后发生小脑缄默综合征的个体相对于未发生者，其病变会优先与以下两者相交：（i）小脑传出通路；（ii）先前生成的小脑缄默综合征“病变-症状图谱”。分析是按照预先注册的假设和分析方法（https://osf.io/r8yjv/）进行的。我们为这两个假设都找到了支持证据。与未发生小脑缄默综合征的患者相比，发生小脑缄默综合征的患者（ = 10）的病变与小脑传出通路的重叠度更高（科恩d值 = 0.73， = 0.05），与小脑缄默综合征病变-症状图谱的重叠度也更高（科恩d值 = 1.1， = 0.004）。这些结果强化了病变位置与发生小脑缄默综合征风险之间的关联，并证明了跨队列的普遍性。这些发现可能有助于为儿科小脑肿瘤的最佳手术方法提供参考。
