1、病 例 讨 论,刘彩燕北京协和医院神经科2013-4,*,M,43y ,建筑工程师主诉:精神行为异常、行动迟缓4月余。,现病史,2012-3 无明显诱因睡眠增多,寡言少语,当时未在意,病情逐渐加重.2012-5 出现反应迟钝,性格改变、异常行为,如对待时孩子方式较前改变,给孩子喂不应该吃的东西,淡漠,记忆力下降,常回忆不起前几天发生的事情,开车忘记拉手刹,行走缓慢,需要他人搀扶。无头痛、头晕、肢体麻木、肢体无力、复视。无发热。二便失禁，食欲可，睡眠较前增多。,现病史,2012-6 就诊于外院 ,血常规、肝肾功、甲功及甲状腺抗体正常、同型半胱氨酸、风湿三项均正常。头MRI：双侧基底节、放射冠、半
2、卵圆中心对称性病灶。腰穿： 白细胞 蛋白 6-8 6 35 6-16 10 40脑脊液结核抗体、囊虫抗体、墨汁染色阴性。甲功3（-）给予抗病毒治疗，无效，病情持续进展。,既往史,2型糖尿病2年，严格饮食控制，素食为主，较少食用肉类，未服药，血糖控制好，体重下降明显。,个人史,大量饮酒史十余年，每日半斤至一斤白酒，5次/周，近2年，半斤/日，3次/周。,家族史（-）。,神经科查体,神清，淡漠，声音低微，反应迟钝。可见双手摸索动作。时间、地点、人物定向力可，记忆力下降，回答不上昨晚饮食；计算力下降，93-7=？。MMSE 14分。颅神经(-)。眼球活动正常。四肢肌力5-级，腱反射对活跃，双侧bab
3、inski（-）、chaddock（+）。感觉（-）。共济（-）。步态异常，步基宽，小碎步，向后倾倒，Romberg征(+)。,问 题,定位诊断?定性诊断考虑哪些?需要进一步做哪些检查?,讨 论,（入院时）定位诊断,临床双侧丘脑：睡眠 语言 认知功能减退 锥体外系 锥体束 皮层下白质自主神经系统,影像学头MRI：双侧基底节半卵圆中心对称性病灶,边缘模糊,定性诊断：（入院时）,营养代谢:Wernick脑病 桥外髓鞘溶解症中毒性：次氯酸等感染性：进行性多灶性白质脑病血管性：Galen静脉血栓形成肿瘤性：淋巴瘤,入院后辅助检查,常规检查：血常规、生化全项、凝血、感染6项、甲功2、甲功3正常。血叶酸、
4、VitB12、正常。血免疫指标：ANA+dsDNA、ENA、ANCA、RA、自身抗体谱均正常。肿瘤指标筛查：（-）毒物筛查：正常。腰穿：压力为170mmH20，脑脊液常规（-），脑脊液生化：Glu4.8mmol/L,余（-），OB、GM1、MBP、HU-YO-RI未见异常。TORCH10项、RPR、TPPA未见异常。,头MRI增强,问 题,定性诊断考虑哪些?还需要做哪些辅助检查?,讨 论,DSA：大脑大静脉动静脉瘘，动脉血供来自双侧颈内动脉系统、左颈外动脉系统及大脑后动脉，大脑大静脉血流逆向充盈，大脑内静脉及基底静脉、直窦显影欠佳。,问题,诊断?病情全貌解释?,结 论,诊断,Bilateral
5、 basal gangalial and white matter lesions,Dural arteriovenous fistula,Figure 1. Distribution of parenchymal lesions in relationship to occluded venous sinuses. Each illustration is accompanied by a list ofoccluded sinuses. SSS indicates superior sagittal sinus; StS, straight sinus; TS, transverse si
6、nus; SS, sigmoid sinus; vG, vein of Galen;iCV, isolated cortical vein; ICVs, internal cerebral veins.,(Stroke. 2009;40:1509-1511.),Dural Arteriovenous Fistulaand CVST,The relationship between the 2 entities is rather complex, because (1) dural fistulas can be a late complication of persistent dural
7、sinus occlusion with increased venous pressure, (2) the fistula can close and cure if the sinus recanalizes, and (3) a preexisting fistula can be the underlying cause of CVT. The exact frequency of dural fistula after CVT is not known because there are no cohort studies with long-term angiographic i
8、nvestigation.,头MRV:大脑内静脉、Galen静脉、直窦未显影头MRA:动静脉瘘,DSA证实,大脑内静脉、Galen静脉、直窦血栓形成,慢性进展病程无颅压升高无头痛血D-dimer正常,硬脑膜动静脉瘘,？,诊断,Bilateral basal gangalial and white matter lesions,Dural arteriovenous fistula,治疗及随诊,治疗,动静脉瘘栓塞+华法令抗凝,随诊病情稳定,文 献 复 习,1、DAVF的定义、分类2、常见的临床表现3、MRI的各种征象4、本例的提示,DAVF的定义和分类,定义：由硬脑膜动脉供血，引流至静脉窦或脑膜
9、静脉的动脉静脉畸形分类：type I ：located in the main sinus with antegrade flowType II: in the main sinus with reflux into the sinus(IIa), cortical veins(IIb),or both(IIa+IIb)Type III: with direct cortical venous drainage without venous ectasiaType IV: with direct cortical venous drainage with venous ectasiaType
10、V: with spinal venous drainage- Radiology 1995;194:671-680,The commonly recognized initial symptoms of a DAVF,tinnitus headacheproptosisdecreased cognitive function neurologic deficits associated with intracranial hemorrhage,Various MRI findings in DAVF,Flow void clusterEngorged ophthalmic vein/prop
11、tosisWhite matter hyperintensityIncranial hemorrhageDilated leptomeningeal or medullary vesselsVenous pouchLeptomeningeal or medullary vascular enhancement-AJNR 2005;26:2500-7,血管流通影、血肿、眼静脉充盈、脑白质异常信号,眼静脉充盈、血管异常增强影,类似本例的文献报道,Case1 Rapidly progressive dementiaCase2 Progressive dementia + Pakinsons syndromeCase3 Pure progressive dementia,Case 1: Rapidly progressive dementia,Case 2: Progressive dementia + Pakinsons syndrome,Case3:Pure dementia,本例的提示,迅速进展的痴呆,脑白质病变,影像学的蛛丝马迹,勿忘DAVF！,谢谢!,
