Setia Vikrant, Zeya Monirah, Srivastava Arvind Kumar, Jagetia Anita
Department of Neurosurgery, Geetanjali Medical College and Hospital, Udaipur, Rajasthan.
Department of Neurosurgery, G.B. Pant Institute of Post Graduate Medical Education and Research, New Delhi, India.
Asian J Neurosurg. 2025 Jan 13;20(2):260-268. doi: 10.1055/s-0044-1801404. eCollection 2025 Jun.
Cerebellar mutism syndrome (CMS) is a postoperative syndrome of decreased speech seen in children associated with neurobehavioral abnormalities, the incidence of which is up to 40%.
To evaluate pediatric patients with posterior fossa tumors for incidence, clinical characteristics, pathophysiology, risk factors, and neuroradiographic features of this syndrome.
The study included 60 pediatric patients with a posterior fossa tumor who underwent surgery by a telovelar approach. Detailed pre- and postoperative clinical and radiological evaluations were done. Patients with CMS were analyzed and compared with those without mutism to find risk factors for CMS. The presentation and characteristics of cerebellar mutism were studied along with the following risk factors:Clinical-age, sex, cranial nerve deficit, and adjuvant treatment.Radiological-tumor location, hydrocephalus, brainstem invasion, extent of tumor resection, peduncular and brainstem edema, and atrophy of posterior fossa structures.Pathological-histopathology of tumor.The preoperative, immediate postoperative, and 1-year postoperative imaging results were reviewed to assess the neuroradiographic features in the two groups.
The incidence of this syndrome was 20%. The mutism was accompanied by some neurobehavioral abnormalities ( -value = 0.05). The most significant finding was the presence of a period of cerebellar dysarthria after the resolution of the muteness ( -value < 0.001) in all cases. Brainstem and related structures' involvement was the most significant risk factor ( -value = 0.03). The presence of brainstem and peduncular edema in the immediate postoperative period ( -value = 0.04) and gross atrophy of posterior fossa structures at 1 year ( -value = 0.01) showed significance toward the development of CMS. There was delayed neurological recovery in patients with CMS with a poor Glasgow Outcome Score at 1 year of follow-up.
The clinical presentation of this syndrome in context with neuroradiographic features suggests that it results from transient impairment of the afferent and/or efferent pathways of dentate nuclei that are involved in initiating complex volitional movements and are associated with brainstem involvement of tumor and poor functional outcome.
小脑缄默综合征（CMS）是儿童术后出现的一种言语减少综合征，伴有神经行为异常，其发生率高达40%。
评估后颅窝肿瘤患儿中该综合征的发生率、临床特征、病理生理学、危险因素及神经影像学特征。
本研究纳入60例采用经小脑幕上入路手术的后颅窝肿瘤患儿。进行了详细的术前和术后临床及影像学评估。对发生CMS的患儿进行分析，并与未发生缄默的患儿进行比较，以找出CMS的危险因素。研究小脑缄默的表现和特征以及以下危险因素：临床方面——年龄、性别、脑神经功能缺损和辅助治疗；影像学方面——肿瘤位置、脑积水、脑干侵犯、肿瘤切除范围、脑桥和脑干水肿以及后颅窝结构萎缩；病理方面——肿瘤的组织病理学。回顾术前、术后即刻及术后1年的影像学结果，以评估两组的神经影像学特征。
该综合征的发生率为20%。缄默伴有一些神经行为异常（P值 = 0.05）。最显著的发现是所有病例在缄默消失后均出现一段小脑构音障碍期（P值 < 0.001）。脑干及相关结构受累是最显著的危险因素（P值 = 0.03）。术后即刻出现脑桥和脑干水肿（P值 = 0.04）以及术后1年出现后颅窝结构明显萎缩（P值 = 0.01）对CMS的发生具有显著意义。CMS患儿在随访1年时格拉斯哥预后评分较差，神经功能恢复延迟。
该综合征的临床表现结合神经影像学特征提示，它是由齿状核传入和/或传出通路的短暂损伤所致，这些通路参与启动复杂的随意运动，且与肿瘤侵犯脑干及功能预后不良有关。
