Dandy—Walker综合征的诊疗问题

本病主要临床表现和病理改变为头部及后颅窝增大、后颅窝蛛网膜囊肿、小脑蚓部发育不全及四脑室侧孔、中间孔闭塞,为脑积水的病因之一。本病很少见,据本文资料统计,约占所有脑积水病例的4％。一般对本病的手术治疗方法是作侧脑室分流术或后颅窝囊肿切除,但问题并未得到很好解决,术后容易复发。作者通过10例手术治疗总结了经     

Dandy—Walker综合征手术治疗1例

1病例 患儿，男，2岁。因头痛、厌食半月，加重伴呕吐3d入院。患儿为顺产，其母怀孕期间未患任何疾病。查体：智力、发育无异常，头围51cm，神志清楚，双眼球活动自如，落日征（-），颅神经阴性，Romberg征（＋），步态不稳。CT示后颅窝巨大扇形囊肿与四脑室相通，脑干前移，小脑两半球分离向前外移、蚓部缺失，横窦及窦汇上移，幕上脑室对称性扩大；MRI示双侧脑室及第三脑室、中脑导水管、第四脑室极度扩张，其后方小脑皮质菲薄，胼胝体发育不良，中线结构居中。     

Dandy—Walker氏囊肿综合征3例报告

1临床资料例1,男,11岁。因行走不稳,持物颤抖伴头痛、恶心、呕吐2月入院。CT检查示幕上脑室扩大,后颅窝巨大囊肿,与第四脑室相通,小脑扁桃体缺如。入院后行囊肿切除加后颅窝减压术,术中见第四脑室约1/2呈开放状态,未见后髓帆。     

Dandy—Walker综合征1例报告并文献复习

Dandy-Walker综合征(DWS),又名Dandy-Walker畸形,第四脑室中、侧孔闭锁,是胚胎时期脑发育畸形,国内报道较少.本文报告该病1例,并结合文献复习,对DWS进行讨论.     

Psychiatric misdiagnoses in Dandy–Walker variant

Cases of intellectual impairment and aberrant behavior in patients with cerebellar diseases have been described since the early nineteenth century. Here, we report on a patient suffering from Dandy–Walker variant who presented with symptoms of obsessive compulsive disorder and delusional disorder. The current findings emphasize the potential relevance of focal cerebellar lesions as organic correlates of these disorders.     

Atypical psychotic symptoms and Dandy–Walker variant

ABSTRACT

New-onset psychotic symptoms often respond well to antipsychotic treatment; however, symptoms may be difficult to treat when an underlying brain malformation is present. Here, we present a case of atypical psychotic symptoms in the context of a congenital cerebellar malformation (Dandy–Walker variant). The patient ultimately improved with paliperidone palmitate after multiple antipsychotic medication trials (both oral and one long-acting injectable) were ineffective. Neuroimaging may provide valuable diagnostic and prognostic information in cases of new-onset psychosis with atypical features and treatment resistance, even in the absence of neurologic signs and symptoms.     

Dandy walker综合征8例报告

Dandy-Walker综合征(DWS),又称侧孔、正中孔闭锁综合征。国内报告甚少。我科1989年5月～1991年3月曾见8例。1例脑CT、7例磁共振(MRI)证实。今对其临床表现、病理、诊治作一简略探讨。 资料 男7例,女1例。年龄38天～5岁,其中2岁以下6例。5例以脑积水,3例分别以化脑、结脑、双下肢力弱待查入院。表现:前囟大而膨隆、头     

Giant occipital meningocele in an 8-year-old child with Dandy–Walker malformation

Introduction  

The possibility of an association between Dandy–Walker malformation and occipital meningocele is well-known. However, just an overall number of about 40 cases have been previously reported. Giant occipital meningocele has been described only in three newborns. Incidence, pathology, clinical presentation, and proper management of this association are still poorly defined.     

A case series of 12 patients with incidental asymptomatic Dandy–Walker syndrome and management

Purpose

Incidentally detected asymptomatic Dandy–Walker syndrome (DWS) is sparsely reported in literature at extremes of age (from 1 to 75?years) in association with different diseases. Precipitating factors causing DWS in these cases to manifest in late adulthood are still unidentified. We tried to hypothesize the aetiology and the natural course of disease based on review of literature

Material and methods

Twelve cases of asymptomatic DWS were selected retrospectively in this study while being treated for some unrelated disease over a period of 15?years.

Results

All the cases had vermian hypoplasia with sizeable fourth ventricular cyst (more than 3?cm), large posterior fossa and with no or borderline ventriculomegaly on CT/MRI. The age ranged from 1 to 65?years. Five cases presented with head injury and four cases presented with enlarged head size with suboccipital protuberance (noticed in children more than 5?years). Remaining cases presented with either occipital encephalocele or right trigeminal neuralgia or fixed atlantoaxial dislocation. They were asymptomatic for DWS following treatment of the presenting complaints on follow-up of average duration of 4.5?years.

Conclusion

Presentation at extremes of age signifies that slow degenerative changes in communicating channels between fourth ventricular cyst and surrounding basal cisterns may cause asymptomatic DWS to manifest, but cases having good communication between these structures can remain asymptomatic throughout their life.     

手—口—足综合征

作者首次报告2例桥脑出血及中脑梗塞引起的手一口一足综合征(cheiro-oral-pedal syndrome),CT 及MRI 证实病变位于内侧丘系及三叉神经腹侧次级上行束。例1,81岁女性,突然左偏瘫,头痛及构音障碍而入院。血压160/90mmHg。意识清楚,有构音障碍,舌微偏左,左侧偏瘫,双侧掌颏反射阳性,左侧口周、前臂远端和下肢远端感觉异常及减退。     

Ramsay——Hunt氏综合征

Ramsay—Hunt氏(1907)以“膝状神经节疱疹性炎症”报告了一组外耳道疱疹—耳痛—面瘫为特征的三联症,称之为Ramsay—Hunt氏综合征,或亨特综合征(Hum Syndrome)、膝状神经节综合征(ganglion geniculatum syndrome)、耳带状癌疹(herpes zoster oticus),本文报导2例,并结合文献讨论如下。例1.祝××,女,50岁。患者以右耳及右枕都麻木、疼痛起病,发病第3日右外耳道、耳廓出现丘疹和成簇内含澄清液体的水疱。剧烈疼痛。第4日口眼歪斜。查体呈典型右侧周围性面瘫,右舌前2/3味觉丧失,余神经系统未见异常。头部CT、血尿常规、肝肾功、脑脊液常规,生化均正常。例2.张××,男,66岁。患者因慢性肺原性心脏病、阻塞性肺气肿、慢性喘息性支气管炎急发,在当地静点地塞米松和青霉素。半月后自觉左耳内灼痛、麻木。翌日,左耳后,耳壳、外耳道出现丘疹及成堆从绿豆到黄豆大小不等的水疱,剧痛难忍,遂转住我院     

Gerstmann—Straussler综合征

Gerstmann -Straussler (1936 )首先发现本病 ,故名Gerstmann -Straussler综合征 (GSS) ,亦称Gerst mann -Straussler -Scheinker综合征 ,是人类传递性海绵状脑病之一 ,属罕见的家族性神经系统变性疾病。主要症状为小脑性共济失调、智力障碍、锥体束征和下肢肌肉萎缩。病理改变为小脑海绵状变性、神经细胞脱失、星形胶质细胞增生 ,以及散在的淀粉样斑块。为常染色体显性遗传疾病。1　病因和发病机制GSS是人类可传递性的海绵状脑病之一。生物分离和纯化技术的成熟 ,导致朊蛋白感染疾病研制中的新的突破。Prusiner和Bolton等人从羊瘙痒…     

Landau—Kleffner综合征

Landau-Kleffner综合征并不罕见,但由于医务人员对其认识不够,易导致漏诊和误诊,现对该综合征的病因、发病机制、临床表现、诊断、治疗和预后等近期文献作一综合论述.