小脑性缄默综合征的研究进展

正小脑性缄默综合征(cerebellar mutism syndrome,CMS)是儿童后颅窝肿瘤术后的一种严重并发症,Rekate等在1985年首先提出小脑性缄默综合征~([1])。儿童后颅窝手术后CMS的发生率为11%~29%~([2]),特别是髓母细胞瘤术后最高可达39%~([3]),偶尔可见于外伤、脑血管意外和感染性疾病~([4])。其特点是以缄默症为主要表     

儿童后颅窝手术后短暂性小脑缄默症

短暂性小脑缄默症作为儿童后颅窝肿瘤术后的一种并发症早已有报道。但多年来此症的发病机理和解剖学改变均不清楚,而且其究竟是一种纯心理障碍,还是器质性病损也一直存有争论。本文通过一组63例儿童后颅窝术后出现的5例短暂性缄默症与文献资料进行了统计学分析,希望能找出一些该综合症的先兆因素和阐明其发病机理。     

儿童后颅窝肿瘤合并脑积水的治疗及预后影响因素分析

目的 探讨儿童后颅窝肿瘤合并脑积水患者行肿瘤切除术前对于脑积水的治疗是否影响术后脑积水的发生率,并分析术后脑积水发生的相关危险因素.方法 中山大学附属第一医院神经外科自2003年1月至2009年12月行肿瘤切除术治疗后颅窝肿瘤合并脑积水儿童患者63例,其中对术前脑积水行保守治疗43例,手术治疗20例.回顾性分析患者的临床资料并比较术前脑积水保守治疗组和手术治疗组患者术后脑积水的发生率,Logistic回归分析患者的性别、发病年龄、术前Evans指数、肿瘤病理类型、肿瘤切除程度、肿瘤生长部位等因素是否与术后脑积水的发生有关.结果 术后出现脑积水患者22例,其中术前脑积水保守治疗组14例(14/43,32.6%),术前脑积水手术治疗组8例(8/20,40.0%),2组比较脑积水发生率差异无统计学意义(P=0.564);Logistic 回归分析发现患者术前Evans指数、肿瘤生长部位、肿瘤切除程度与肿瘤切除术后脑积水的发生有关(P=0.029,p=0.036,P=0.038).结论 肿瘤切除术前脑积水的治疗并不能改变术后脑积水的发生率:术前Evans指数、肿瘤生长部位、肿瘤切除程度是肿瘤切除术后脑积水发生的危险因素.     

儿童后颅窝肿瘤合并脑积水的治疗及预后影响因素分析

目的 探讨儿童后颅窝肿瘤合并脑积水患者行肿瘤切除术前对于脑积水的治疗是否影响术后脑积水的发生率,并分析术后脑积水发生的相关危险因素.方法 中山大学附属第一医院神经外科自2003年1月至2009年12月行肿瘤切除术治疗后颅窝肿瘤合并脑积水儿童患者63例,其中对术前脑积水行保守治疗43例,手术治疗20例.回顾性分析患者的临床资料并比较术前脑积水保守治疗组和手术治疗组患者术后脑积水的发生率,Logistic回归分析患者的性别、发病年龄、术前Evans指数、肿瘤病理类型、肿瘤切除程度、肿瘤生长部位等因素是否与术后脑积水的发生有关.结果 术后出现脑积水患者22例,其中术前脑积水保守治疗组14例(14/43,32.6%),术前脑积水手术治疗组8例(8/20,40.0%),2组比较脑积水发生率差异无统计学意义(P=0.564);Logistic 回归分析发现患者术前Evans指数、肿瘤生长部位、肿瘤切除程度与肿瘤切除术后脑积水的发生有关(P=0.029,p=0.036,P=0.038).结论 肿瘤切除术前脑积水的治疗并不能改变术后脑积水的发生率:术前Evans指数、肿瘤生长部位、肿瘤切除程度是肿瘤切除术后脑积水发生的危险因素.     

后颅窝肿瘤术后缄默症38例报告

小脑缄默症在 1985年被首先描述 ,为可逆性的综合症 ,多发生于儿童后颅窝肿瘤术后。我院在 1988～ 2 0 0 2年共遇到 38例 ,现将其临床特点介绍如下。1　临床资料1 1　一般资料 :我院自 1988～ 2 0 0 2年共遇到后颅窝肿瘤术后缄默症患者 38例 ,男 2 2例 ,女 16例 ,年龄 2～ 5 8岁 ,平均 9 4岁。儿童 (2～ 13岁 ) 35例 ,占 92 5 % ,潜伏期 0～ 5d ,平均潜伏期 1 4d ,16例术后出现脑干受损症状 ,占 4 5 7% ,31例术后出现小脑蚓部受损症状 ,占 89 5 %。成人 3例 ,潜伏期 0～ 4d,平均潜伏期 2d ,出现术后脑干受损症状及小脑蚓部受损症状者各 1…     

小脑性缄默和继发构音障碍综合征

小脑性缄默是指继发于小脑肿瘤术后的暂时性不能讲话,但发音器官正常,恢复后常伴有构音障碍。本文报道2004年1月至2009年6月收治的8例小脑性缄默症如下：     

颅内压监测下立体定向脑室外引流术在后颅窝肿瘤切除术中的应用

目的探讨颅内压监测下立体定向脑室外引流术对后颅窝肿瘤切除术后的临床应用效果。方法回顾性分析新疆医科大学附属中医医院神经外科2015年1月到2017年8月收治的11例确诊为后颅窝肿瘤患者的临床资料,所有患者术前颅脑CT或MRI均提示伴发不同程度梗阻性脑积水,均在开颅术前行颅内压监测下行立体定向脑室外引流术。结果 11例患者未发生肿瘤切除术前脑积水所致脑疝危象,其中有10在肿瘤切除术后2周复查CT见后颅窝水肿消退、脑室大小正常,康复出院; 1例因肿瘤侵犯脑干无法全切,术后需在适当延长脑室外引流管的前提下,充分置换脑脊液后症状缓解后出院。11例患者均未发生术后伤口脑脊液漏或颅内感染。结论采用颅内压监测下立体定向脑室外引流术对后颅窝肿瘤并脑积水,可以降低术前发生脑疝的危险,为患者顺利完成肿瘤切除术创造手术机会,可有效降低死亡率。     

内镜第三脑室底造瘘术在儿童后颅窝肿瘤所致梗阻性脑积水中的应用分析

目的探讨内镜第三脑室底造瘘术(ETV)在儿童后颅窝肿瘤所致梗阻性脑积水中的应用效果及并发症情况。方法回顾性分析2013年9月至2021年9月上海交通大学医学院附属上海儿童医学中心神经外科收治的93例后颅窝肿瘤患儿的临床资料。所有患儿均存在急性梗阻性脑积水所致的颅高压症状, 并于二期肿瘤切除术前行一期ETV。分析ETV的术后疗效、并发症发生率, 以及肿瘤切除术后的脑积水复发率。结果 98.9%(92/93)的患儿ETV成功实施, 且术后颅高压症状缓解, 1例患儿因术中出血导致行ETV失败。术后并发症(1例发生颅内感染)的发生率为1.1%(1/92), 经抗感染治疗后好转。肿瘤切除术后的脑积水复发率为6.5%(6/92), 经再次手术置入脑室-腹腔分流管, 脑积水均获得缓解。结论小儿后颅窝肿瘤合并梗阻性脑积水需急诊缓解颅高压时应首选ETV, 其并发症少, 且肿瘤切除术后的脑积水复发率低。     

小脑性缄默4例报告及文献复习

目的探讨后颅窝手术后小脑性缄默的临床特点及其形成的病理生理机制。方法回顾性总结分析我科4例和英文文献报告的137例共141例后颅窝手术后出现的小脑性缄默病例。结果儿童病例127例,占90.1%。手术病变位于小脑蚓部者125例(88.7%)。小脑性缄默均为暂时性,发生的平均潜伏期和持续时间分别为1.7d和56.2d。结论小脑性缄默多见于儿童小脑蚓部肿瘤手术后其发生机制可能与小脑齿状核丘脑腹外侧核运动区和辅助运动区之间的纤维联系的直接或间接损害有关。     

引流术后儿童后颅窝肿瘤瘤内出血的临床治疗(附2例临床分析)

目的探讨后颅窝肿瘤合并梗阻性脑积水行分流术后所致瘤内出血的诊断和治疗经验。方法回顾性分析上海儿童医学中心2013年1月至2014年6月收治后颅窝肿瘤合并脑积水患儿临床资料,均为行分流术而后发生瘤内出血病例。结果该院收治的后颅窝肿瘤合并脑积水患儿11例,其中2例患者出现术后瘤内出血。出血后短期内1例接受手术,1例予以保守治疗,后期病理分别为未成熟畸胎瘤(II级)和胶质神经元肿瘤(Ⅰ级)。结论后颅窝肿瘤常合并梗阻性脑积水,先行分流术后,发生瘤内出血较为罕见。分流术后病情突然恶化,需考虑瘤内出血可能,应急诊行头颅CT检查,并根据具体情况选择最佳治疗措施。     

Development of the projection from the nucleus of the brachium of the inferior colliculus to the superior colliculus in the ferret

Neurons in the deeper layers of the superior colliculus (SC) have spatially tuned receptive fields that are arranged to form a map of auditory space. The spatial tuning of these neurons emerges gradually in an experience-dependent manner after the onset of hearing, but the relative contributions of peripheral and central factors in this process of maturation are unknown. We have studied the postnatal development of the projection to the ferret SC from the nucleus of the brachium of the inferior colliculus (nBIC), its main source of auditory input, to determine whether the emergence of auditory map topography can be attributed to anatomical rewiring of this projection. The pattern of retrograde labeling produced by injections of fluorescent microspheres in the SC on postnatal day (P) 0 and just after the age of hearing onset (P29), showed that the nBIC-SC projection is topographically organized in the rostrocaudal axis, along which sound azimuth is represented, from birth. Injections of biotinylated dextran amine-fluorescein into the nBIC at different ages (P30, 60, and 90) labeled axons with numerous terminals and en passant boutons throughout the deeper layers of the SC. This labeling covered the entire mediolateral extent of the SC, but, in keeping with the pattern of retrograde labeling following microsphere injections in the SC, was more restricted rostrocaudally. No systematic changes were observed with age. The stability of the nBIC-SC projection over this period suggests that developmental changes in auditory spatial tuning involve other processes, rather than a gross refinement of the projection from the nBIC.     

Mechanisms of the suppression of the nociceptive reflex of the opening of the mouth during stimulation of the structures of the limbic brain

The comparative effectiveness of the inhibitory influence of tetanic stimulation of hypothalamus, amygdala and limbic cortex on EMG-response of m. digastricus evoked by electrical stimulation of tooth pulp nociceptive afferents was studied in cats anesthetized with a mixture of chloralose and nembutal. It was found that inhibition of the EMG-component of the jaw-opening reflex is most pronounced in case of stimulation of medial and lateral region of the hypothalamus, the inhibitory effect of central and medial nuclei of the amygdala is less pronounced and the effect of the limbic cortex is the weakest. It was shown that the mechanism of the antinociceptive effect of tetanic stimulation of the hypothalamus is not related to the concomitant increase of the blood pressure. After stabilization of the blood pressure the suppressive effect of the hypothalamus remains without changes, that points out to a direct, primary, not baro-afferent mechanism of the inhibition of the activity of nociceptive neurons of the trigeminal sensory nuclei. Noradrenaline, injected intravenously, induced a large increase of the blood pressure accompanied by a pronounced inhibition of the pain reflex. Angiotensin causes the same degree of blood pressure elevation without changes in the amplitude of the EMG-response of the pain reflex. Hypothalamic and noradrenergic mechanisms for control of pain sensitivity are discussed.     

The organization of the connections between the cortex and the claustrum in the monkey

The distribution of labelled cells and of extracellular granules in the claustrum has been studied after injections of horseradish peroxidase in several areas of the neocortex. The frontal and parietal lobes are related to the anterior and posterior halves respectively of the claustrum, and the occipital and temporal cortex to the posterior and inferior margins. Parts of the claustrum related to areas of the cortex in the frontal lobe overlap considerably in the antero-posterior dimension with parts related to widely separated but interconnected areas of the parieto-temporal cortex. Areas of cortex within one lobe which are interconnected are related to parts of the claustrum which overlap in the dorsoventral dimension.     

Investigating the construct validity of the SPAI-C: comparing the sensitivity and specificity of the SPAI-C and the SAS-A

Investigates the construct validity of the Social Phobia and Anxiety Inventory for Children (SPAI-C) by comparing its sensitivity and specificity with another self-report measure of social anxiety, the Social Anxiety Scale for Adolescents (SAS-A). Participants were 252 adolescents (124 males and 128 females) 13-17 years old. Adolescents completed the SPAI-C and the SAS-A and were interviewed using the Anxiety Disorders Interview Schedule for DSM-IV: Child Version (ADIS-IV:C). Parents were also interviewed and composite diagnoses were formed. Youth were classified as socially phobic or non-anxious based on these composite diagnoses. By comparing clinical cutoff scores with diagnostic group classification, the sensitivity and the specificity of the SPAI-C and SAS-A were compared. Results indicated that the SPAI-C was a more sensitive measure than the SAS-A (61.5% vs. 43.6%) providing evidence of the scale's construct validity. The two measures were similar with regard to specificity (82.7% for both). Implications of these results for assessment and research are discussed.