 一日，葱花给大汉提供了一个病例，让大汉猜猜看是什么病。
A 65-year-old man with a 3-month history ofprogressive cognitive decline and falls 65岁的男性患者，渐展性认知衰退和跌倒3个月。 AxialNECT (A) at presentation shows bithalamic low attenuation and swelling,indicating edema. 轴位NECT(A)显示双丘脑低密度和肿胀，表明存在水肿。 Axial FLAIR (B) at presentation confirms bithalamic vasogenicedema. 轴位FLAIR (B)证实了双侧丘脑血管源性水肿。 大汉想了想影响双侧丘脑的疾病，答：Percheron动脉闭塞？CVST？胶质瘤？葱花的答案是NO！大汉又想了想公众号曾经写过的进展性痴呆萌妹子课堂——萌妹子说文解字辩痴呆（点击查看相关内容），赶紧看了看，还是不明白，最后还是向葱花问个明白。 SubsequentTOF MRA MIP 3D (C) performed after failure to improve with anticoagulationshows abnormal arterial flow signal within a dilated and tortuous supravermianvein (C, white arrow) near distal left posterior cerebral artery (C, blackarrow). 抗凝治疗后临床未能改善，随后的MRA MIP 3D(C)发现在左大脑后动脉(C，黑色箭头) 远端附近的上蚓静脉(C，白色箭头) 有异常动脉血流信号。 3D-VRreconstruction from a left vertebral artery rotational DSA (D) shows a torcularregion DAVF supplied by the posterior meningeal artery branches (thin whitearrow. Fistulous point-black arrow, straight sinus-large white arrow). 从左椎动脉旋转3d-VR重建DSA(D)显示由脑膜后动脉分支(细白箭头)供血的DAVF区域。（瘘点-黑箭头，直窦-大白箭头）。 Postembolizationunsubtracted frontal control image (E) shows liquid embolization material(arrow) filling the fistula. 栓塞后正位像(E)显示液体栓塞材料(箭头)填充于瘘。 Three-monthpostembolization follow-up axial FLAIR (F) shows complete normalization of thebithalamic signal abnormalities. The patient showed full recovery of hiscognitive function. 栓塞后三个月的随访轴位FLAIR (F)显示之前有异常信号的双侧丘脑完全正常。病人的认知功能完全恢复。 诊断：DURAL ARTERIOVENOUS FISTULA (DAVF)
Background: Potentially reversible cause of progressivedementia Easily missed without vascular imaging (asin this case) Requires surgical disconnection; in thislocation, conventional ligation is difficult and endovascular embolization ispreferred. 背景： 可逆转的进展性痴呆的原因 无血管成像(如本例)的很容易被忽略； 需要手术切除，在此位置，结扎困难，血管内栓塞是首选。 ClinicalPresentation: Progressive cognitive decline, includingmemory disturbance; Montreal Cognitive Assessment score of 10/30 Weight loss and ataxia 临床表现: 进行性认知功能下降，包括记忆障碍，MOCA评分10分。 体重下降和共济失调 KeyDiagnostic Features: Symmetrical bithalamic edema on CTconfirmed by MRI (FLAIR hyperintensity) No diffusion restriction (cytotoxic edema)on DWI/ADC and no venous abnormality Abnormal arterial flow signal withintortuous peri-vein of Galen vascular structure demonstrated by MRA Formal DSA confirms arteriovenous shuntfrom left-sided posterior meningeal arteries into distended collateral veinsand vein of Galen Embolization material fills fistulousconnection Complete resolution of FLAIR signalabnormality at 12 weeks postembolization 关键诊断特征： 通过CT/MRI证实的对称性双侧丘脑水肿（FLAIR高信号） DWI/ADC无弥散受限（细胞毒性水肿），无静脉异常 常规DSA证实从左侧脑膜后动脉到Galen静脉及其侧枝静脉的动静脉分流 栓塞材料填充于瘘 栓塞后12W FLAIR异常信号完全消失 DifferentialDiagnoses: Vascular: Acute stroke secondary to artery of Percheron occlusion: woulddemonstrate high DWI and low ADC signals (restricted water diffusion) Infection: Viral encephalitis includingWest Nile and CJD: expect relevant travel history +/- cortical signalabnormality +/- callosal splenium lesion Metabolic: Osmotic myelinolysis: bithalamicinvolvement is unusual; abnormal signal often includes pons and water diffusioncan be restricted. Inherited metabolic: Fabry disease(lysosomal storage disease) and Leigh syndrome (mitochondrial disease):diffusion restriction expected. May show the T1 hyperintense pulvinar sign. Neoplasm: Diffuse low-grade astrocytoma:vascular imaging would be normal; otherwise difficult to differentiate 鉴别诊断: 血管性：继发于Percheron动脉闭塞的急性卒中：影像学显示DWI高信号和ADC低信号(水分子弥散受限) 感染性：包括西尼罗在内的病毒性脑炎和CJD：应有相关的旅行史+ / -皮质信号异常+ /- 胼胝体压部病损 代谢性：渗透性脱髓鞘：双侧丘脑受累不常见，异常信号常见于脑桥，水分子弥散受限 遗传性：法布里病(溶酶体贮积病)和Leigh综合征(线粒体疾病)：可有弥散受限，丘脑枕部T1高信号 肿瘤性：弥漫性低级别星形细胞瘤:血管成像是正常的；否则很难区分 Treatment: Disconnection of arteriovenous shunt(surgical or endovascular using liquid embolic via transarterial or transvenousapproach) Increasing endovascular management withimproved neurologic outcome Follow-up imaging is recommended to reviewfor resolution of bithalamic edema and to exclude any residual orrecanalization of fistula. This can be restricted to short-term if completeocclusion is confirmed. Clinical improvement can occur within daysof definitive treatment. In this case, dementia completely resolved.
治疗: 切断动静脉吻合(手术或通过经动脉或经静脉入路使用流体血管内栓塞术) 越来越多的血管内治疗能改善神经系统的结局 影像随访可以观察双丘脑水肿的恢复情况，排除瘘管的残余或再通。 进行在正确治疗后的几天内，临床症状就改善。本患者，痴呆完全恢复。 |
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