Mitral insufficiency with congenital double-orifice mitral valve in an elderly patient.

We describe the case of a 79-year-old woman with mitral insufficiency and a double-orifice mitral valve (DOMV), discovered by echocardiography. Transthoracic echocardiography showed two insufficiency jets. Transesophageal echocardiography revealed a DOMV. Each orifice was provided with a subvalvular apparatus. No associated congenital abnormalities were present. Our case demonstrates that even in elderly patients with a double regurgitant jet, DOMV should be suspected and assessed by transesophageal echocardiography.     

Cavernous angiomas of the spinal district: surgical treatment of 11 patients

Cavernous angiomas, also called cavernous malformations or cavernomas, are vascular hamartomas accounting for 3–16% of all angiomatous lesions of the spinal district. Although histologically identical, these vascular anomalies may exhibit different clinical behavior and radiological features, depending on their location, hinting at different managements and therapeutic approaches. The authors report 11 cases of symptomatic spinal cavernous angiomas diagnosed and surgically treated over the past 18 years. Age of patients ranged from 15–75 years; males outnumbered females. Three patients had vertebral cavernous malformations, secondarily invading the epidural space; two had pure epidural lesions; two patients had intradural extramedullary lesions, and four intramedullary lesions. Surgical removal was completely achieved in four patients with intramedullary lesions, in two with subdural extramedullary lesions, and in one with a pure epidural lesion. Subtotal excision of another one epidural and three vertebral cavernous angiomas was followed by radiotherapy. There was no morbidity related to surgery; the mean follow-up was 2 years. The outcome was excellent in two cases, good in six, and unchanged in the other three. The authors discuss the different modalities of treatment of these vascular lesions variously placed along the spine.     

头面部复杂动静脉畸形的栓塞治疗

目的　研究经导管栓塞技术在头面部动静脉畸形治疗中的应用。方法　 3 0例头面部动静脉畸形患者共接受 40次栓塞治疗。其中使用国产TH胶栓塞者为 18例 2 0次 ,使用进口PVA微粒栓塞者为 12例 2 0次。结果　 3 0例患者均成功实行瘤灶供血动脉栓塞术。所有患者栓塞术后临床症状明显改善 ,如瘤体缩小甚至消失 ,皮温降低 ,搏动感和血管杂音减弱或消失等。术后随访3～ 6个月 ,12例复发。全部病例均无严重并发症。结论　对于头面部动静脉畸形的患者来说 ,经导管栓塞术是一种较好的非手术治疗方法 ,在栓塞剂的选择方面 ,则以PVA微粒栓塞更为理想。     

Experimental Hydrocephalus in Suckling Hamster Induced by Myxovirus Infection

Abstract This study was undertaken to elucidate the pathogenesis of the hydrocephalus and aqueductal stenosis induced by intracerebral mumps virus inoculation in suckling hamsters.
Mild ventricular dilatation became apparent after 5 days of inoculation. Focal denuding of the ependymal layer and subsequent aqueductal stenosis were observed by 14 days after inoculation. The virus antigen was detected not only in the ependymal cells and choroid plexus, but also in some neurons in the cerebral cortex, hippocampus, midbrain and cerebellum. In the cerebral aqueduct, the orderly arrangement of the cilialy clusters was destroyed on the 5th day after inoculation. After 10 days, proliferation of GFAP positive cells was noticed around the cerebral aqueduct and subsequently caused aqueductal stenosis. In the advanced state of hydrocephalus, the cerebellum was displaced downward and showed an elongated, atrophic and sleevelike structure similar to the Arnold-Chiari malformation. It was suggested that the extensive damage of the ependymal cilia may account for early ventricular dilatation, and subsequent aqueductal stenosis with glial proliferation is the main cause of the advanced hydrocephalus. It has not yet been determined whether the mumps virus can pass through the human placenta or not. If it can, however, our results strongly suggest that mumps virus infection in the human fetus will cause congenital hydrocephalus.     

Transjugular percutaneous inoue balloon mitral commissurotomy in a patient with inferior vena cava obstruction after liver transplantation.

Percutaneous transvenous mitral commissurotomy was performed successfully via the transjugular approach in a patient with severe rheumatic mitral stenosis and obstruction of the inferior vena cava due to prior liver transplantation. This case demonstrates the advantage of the jugular approach in patients with difficult anatomy.     

Ulnar distribution of reflex sympathetic dystrophy due to compression of the brachial plexus by a primary venous malformation

An atypical variant of reflex sympathetic dystrophy (RSD) is presented in a 45 year old female with a vascular malformation of the right arm and chest wall. The mechanism was thought to be compression of the brachial plexus by the malformation. The unique scintigraphic features of this presentation of RSD in the ulnar arterial distribution are illustrated.     

Arteriovenous malformations that rupture during pregnancy: a management dilemma

Summary Intracranial haemorrhage due to rupture of an arteriovenous malformation (AVM) during pregnancy is a rare but serious condition that warrants prompt recognition. Once the diagnosis is made, the management is primarily based on neurosurgical rather than obstetric considerations. Due to its rarity, no definitive guidelines exist, and the best time to perform elective surgery (i.e., at presentation or at completion of the pregnancy) is ill-defined. This report describes three patients recently treated at our institution who had AVMs that ruptured during pregnancy. These cases well summarize the difficulties encountered in treating such patients. The diagnostic as well as the therapeutic implications of this condition are discussed.     

Clinical impact of CCM mutation detection in familial cavernous angioma

Introduction and background A 3-year-old Bosnian girl with a large symptomatic brainstem and multiple supratentorial cavernous angiomas, who underwent neurosurgical treatment, is presented. As multiple cavernomas are more common in familial cases, genetic analyses and neuroradiological imaging were performed in the patient and her parents to see whether there was any evidence for inheritance. This information is important for genetic counseling and provision of medical care for at-risk relatives. Currently, no recommendation is available on how to manage these cases.Results Genetic analyses demonstrated a novel CCM1 frameshift mutation (c.1683_1684insA; p.V562SfsX6) in the child and the asymptomatic 27-year-old mother. Sensitive gradient-echo magnetic resonance imaging of the mother revealed multiple supratentorial lesions, whereas analogous imaging of the father showed no pathological findings.Conclusion This case exemplifies that seemingly sporadic cases with multiple lesions might well be hereditary and that presymptomatic genetic testing of family members may identify relatives for whom clinical and neuroradiological monitoring is indicated.     

脑动静脉畸形实验动物模型的建立

脑动静脉畸形是一种比较常见的严重威胁人们生命安全和生存质量的脑血管疾病，目前人们主要是通过尸体解剖以及CT、MRI等影像学的方法认识。近年来，学者们应用各种动物模型来研究脑动静脉畸形的血流动力学，病理生理学以及脑动静脉畸形栓塞材料的评估，栓塞后临床疗效评价，放射照射剂量对脑动静脉畸形的影响等。本文就脑动静脉畸形实验动物的常用种类，动物模型的建立方法以及动物模型的实际应用进行了综述。     

经皮血管内栓塞颅内动静脉畸形（附38例治疗效果观察）

经皮血管内采用氰基丙烯酸异丁脂（ＩＢＣＡ）栓塞治疗３８例颅内动静脉畸形（ＡＶＭ）病人。其中８例达到１００％完全栓塞，栓塞面积大于７５％的１０例，大于５０％者１１例；余下９例栓塞面积小于５０％，１９例以出血为首发症状，１１例以癫痫为首发症状，６例神经功能障碍，２例以头痛为主要症状。栓塞术后发生并发症５例，占１３％，无死亡病例。本文讨论了超选插管技术问题、Ａｍｙｔａｌ试验假阴性、导管粘连及正常灌注压突破。