Robertson Patricia L, Muraszko Karin M, Holmes Emiko J, Sposto Richard, Packer Roger J, Gajjar Amar, Dias Mark S, Allen Jeffrey C
Department of Pediatrics, University of Michigan Health System, Ann Arbor, Michigan 48109-0203, USA.
J Neurosurg. 2006 Dec;105(6 Suppl):444-51. doi: 10.3171/ped.2006.105.6.444.
Cerebellar mutism syndrome (CMS) is a unique postoperative syndrome typically arising 1 to 2 days after resection of a midline posterior fossa tumor; it consists of diminished speech progressing to mutism, emotional lability, hypotonia, and ataxia. Most descriptions have been limited to small institutional series using a retrospective chart review methodology.
The authors incorporated a CMS questionnaire in two large clinical trials (Children's Cancer Group [CCG] 9931, treatment for high-risk medulloblastoma/primitive neuroectodermal tumor; and CCG/Pediatric Oncology Group [POG] A9961, treatment for average-risk medulloblastoma) to prospectively survey for incidence, severity, and possible causes of CMS in children with newly diagnosed medulloblastoma. Information pertaining to 450 of the 463 patients enrolled in the studies was available for review (82 patients in CCG 9931, and 368 patients in CCG/POG A9961). Cerebellar mutism syndrome occurred in 107 (24%) of 450 children. Symptom intensity was judged to have been severe in 43%, moderate in 49%, and mild in 8% of these 107 patients. Mutism and ataxia were the features most frequently judged as severe. In both cohorts, preoperative brainstem invasion was the only feature that correlated with risk of CMS. One year after diagnosis, nonmotor speech/language deficits, neurocognitive deficits, and/or ataxia persisted in a significant fraction of patients.
Nearly one quarter of patients who underwent resection of a medulloblastoma developed symptoms of CMS, of which 92% were judged to be of moderate or severe intensity. Brainstem invasion by tumor was the only risk factor that correlated positively with CMS occurrence; there was a negative correlation with cerebellar hemisphere tumor location. As more radical resections are attempted for medulloblastoma, the potential for increased morbidity must be carefully weighed against prognostic factors, especially in patients with brainstem invasion.
小脑缄默综合征（CMS）是一种独特的术后综合征，通常在切除后颅窝中线肿瘤后1至2天出现；其表现为言语减少逐渐发展为缄默、情绪不稳定、肌张力减退和共济失调。大多数描述仅限于使用回顾性病历审查方法的小型机构系列研究。
作者在两项大型临床试验（儿童癌症组[CCG]9931，高危髓母细胞瘤/原始神经外胚层肿瘤的治疗；以及CCG/儿科肿瘤学组[POG]A9961，中危髓母细胞瘤的治疗）中纳入了一份CMS问卷，以前瞻性调查新诊断髓母细胞瘤患儿中CMS的发生率、严重程度及可能病因。对纳入研究的463例患者中的450例患者的信息进行了审查（CCG 9931中有82例患者，CCG/POG A9961中有368例患者）。450例儿童中有107例（24%）发生了小脑缄默综合征。在这107例患者中，43%的症状强度被判定为严重，49%为中度，8%为轻度。缄默和共济失调是最常被判定为严重的特征。在两个队列中，术前脑干受侵是与CMS风险相关的唯一特征。诊断后一年，相当一部分患者持续存在非运动性言语/语言缺陷、神经认知缺陷和/或共济失调。
近四分之一接受髓母细胞瘤切除术的患者出现了CMS症状，其中92%被判定为中度或重度。肿瘤侵犯脑干是与CMS发生呈正相关的唯一危险因素；与小脑半球肿瘤位置呈负相关。随着对髓母细胞瘤尝试进行更彻底的切除，必须仔细权衡发病率增加的可能性与预后因素，尤其是对于脑干受侵的患者。
