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儿童后颅窝肿瘤切除术后的缄默症和假性球麻痹症状:发病率及病理生理学

Mutism and pseudobulbar symptoms after resection of posterior fossa tumors in children: incidence and pathophysiology.

作者信息

Pollack I F, Polinko P, Albright A L, Towbin R, Fitz C

机构信息

Department of Neurosurgery, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, Pennsylvania, USA.

出版信息

Neurosurgery. 1995 Nov;37(5):885-93. doi: 10.1227/00006123-199511000-00006.

Abstract

MUTISM AND A variety of other neurobehavioral symptoms have been reported anecdotally after the removal of posterior fossa mass lesions. To determine the incidence and clinical spectrum of this syndrome, a detailed review was performed of patients undergoing resection of infratentorial tumors at our institution during the last 9 years; 12 of 142 patients (8.5%) manifested this syndrome, the largest series of such patients reported to date. Each child had a lesion that involved the vermis; seven had medulloblastomas, three had astrocytomas, and two had ependymomas. The incidence among children with vermian neoplasms was 13%. Ten children underwent division of the inferior vermis during tumor resection, and three had a superior vermian incision; one child underwent both superior and inferior vermian incisions. In 10 children, mutism developed in a delayed fashion postoperatively. The speech disturbance was associated with poor oral intake in 9 children, urinary retention in 5, long-tract signs in 6, and bizarre personality changes, emotional lability, and/or decreased initiation of voluntary movements in all 12. Neuropsychiatric testing, performed in seven children, confirmed impairments not only in speech but also in initiation of other motor activities. Ten children regained normal speech, bladder control, and neurological functioning, other than ataxia and mild dysarthria, within 1 to 16 weeks; two children had significant residual deficits. Characteristically, affect and oral intake returned to their preoperative baseline before the speech difficulties began to resolve. A detailed radiological review of these cases in parallel with 24 cases of vermian tumors without mutism identified only one factor that was significantly associated with the mutism syndrome, bilateral edema within the brachium pontis (P < 0.01). Neither the size of the tumor nor the length of vermian incision was associated with the development of mutism. The clinical features of this syndrome in the context of these imaging findings suggest that the mutism syndrome results from transient impairment of the afferent and/or efferent pathways of the dendate nuclei that are involved in initiating complex volitional movements. The clinical courses of our patients are presented and compared with those of similar cases in the literature in an attempt to evaluate the validity of this hypothesis.

摘要

据传闻,后颅窝肿块病变切除后会出现缄默症及多种其他神经行为症状。为确定该综合征的发病率及临床谱,我们对本机构过去9年中接受幕下肿瘤切除术的患者进行了详细回顾;142例患者中有12例(8.5%)出现了该综合征,是迄今为止报道的此类患者数量最多的系列研究。每个儿童都有累及小脑蚓部的病变;7例为髓母细胞瘤,3例为星形细胞瘤,2例为室管膜瘤。小脑蚓部肿瘤患儿中的发病率为13%。10例儿童在肿瘤切除过程中进行了小脑蚓部下部分离,3例进行了小脑蚓部上部分切开;1例儿童同时进行了小脑蚓部上、下部分切开。10例儿童术后出现延迟性缄默。9例儿童的言语障碍与经口摄入量减少有关,5例与尿潴留有关,6例与长束征有关,所有12例均出现怪异的人格改变、情绪不稳定和/或自主运动启动减少。对7例儿童进行的神经心理学测试证实,不仅言语功能受损,其他运动活动的启动也受到影响。10例儿童在1至16周内恢复了正常言语、膀胱控制及神经功能,但存在共济失调和轻度构音障碍;2例儿童有明显的残留缺陷。其特点是,在言语困难开始缓解之前,情感和经口摄入量已恢复到术前基线水平。对这些病例与24例无缄默症的小脑蚓部肿瘤病例进行详细的影像学回顾,结果仅发现一个与缄默症综合征显著相关的因素,即脑桥臂内双侧水肿(P<0.01)。肿瘤大小和小脑蚓部切口长度均与缄默症的发生无关。结合这些影像学表现,该综合征的临床特征提示,缄默症综合征是由于参与启动复杂随意运动的齿状核传入和/或传出通路的短暂受损所致。本文介绍了我们患者的临床病程,并与文献中类似病例的病程进行比较,以评估这一假说的正确性。

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