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A 52-year-old previously healthy man presented with 8 months of progressive cognitive decline. He complained of months of confusion, fatigue, depression, hypersomnolence, headaches, and, subsequently, urinary incontinence and unsteady gait. His family reported that he spoke of his deceased mother as if she were alive. His executive deficits progressed, leading to termination of his employment and a motor vehicle accident. He was evaluated and treated in Morocco before presenting to our institution for further care.
52岁，男性，既往体健，以进行性认知功能下降8个月入院，主要表现为思维混乱、乏力、抑郁、嗜睡、头痛及尿失禁和步态不稳。母亲已故，自觉依然健在。执行功能受损逐渐加重，导致失业并出现交通事故。在摩洛哥进行评估及治疗后，随转入我院进一步诊治。
Mental status examination was notable for slowed mentation and dyscalculia but was otherwise normal. Motor, sensory, and deep tendon reflex examination results were normal. Cerebellar and gait examination revealed wide-based gait and lower extremity dysmetria. Relevant laboratory evaluation revealed only a C-reactive protein of 1.79 (normal 0–0.99).
简易智力状态检查（MMSE）显示反应迟钝和计算力障碍，余正常。运动、感觉和腱反射检查无异常；小脑和步态检查示宽基底步态和下肢远端辨距不良；实验室检查示C反应蛋白1.79（正常值范围：0-0.99）。
Brain MRI obtained in Morocco 2 months prior to presentation had demonstrated bilateral thalamic T2 hyperintensities and patchy enhancement in the right medial temporal lobe, midbrain, and basal ganglia. A right thalamic biopsy obtained in Morocco had revealed “inflammatory cells” without evidence of malignancy.
入院前2个月在摩洛哥行脑MRI检查，显示双侧丘脑T2高信号和右颞叶内侧、中脑和基底神经节的斑片状强化；右侧丘脑活检示“炎症细胞”浸润，无恶性肿瘤证据。
Questions for consideration 1. How can thalamic injury cause encephalopathy? 2. What is the differential diagnosis for bilateral thalamic MRI abnormalities? 思考问题 1. 丘脑损伤如何引起脑病？ 2. 双侧丘脑MRI异常的鉴别诊断是什么？ The thalamus is a major processing center for the brain that relays motor, sensory, cerebellar, as well as cognitive and limbic inputs. Information regarding cognition and arousal is processed in the mediodorsal, midline, and intralaminar thalamic nuclei. The latter 2 are part of the reticular activating system, which is involved in maintaining consciousness and arousal. Perturbations in these portions of the thalamus can cause confusion, depressed levels of alertness, and coma. Lesions of other structures initially affected on the patient’s MRI, such as the basal ganglia and medial temporal lobe, can produce similar symptoms; however, only bilateral thalamic injury was redemonstrated on our own imaging.
丘脑是大脑主要处理中心，传递运动、感觉、小脑以及认知和边缘系统的冲动。认知和觉醒的信息处理在背内侧、中线和板内侧丘脑核中。后两者是网状激活系统的一部分，参与维持意识和觉醒。累及丘脑的这些结构可造成思维混乱、警觉性低下甚至昏迷。其他结构的损害影响MRI的显示，例如基底神经节和颞叶内侧，可产生类似的症状;下面再次示例仅双侧丘脑损害影像学表现。
The differential diagnosis for thalamic lesions is broad. Etiologies for bilateral thalamic MRI abnormalities include neoplastic (glioma or lymphoma), hereditary, metabolic and toxic (Wernicke encephalopathy, osmotic myelinolysis, pantothenate kinase deficiency, Wilson disease, liver disease, hypoxic ischemic encephalopathy, carbon monoxide poisoning), infectious (West Nile virus or other flaviviruses, Creutzfeldt-Jakob disease, toxoplasmosis), inflammatory (neuro-Beh?et disease), and vascular (occlusion at the top of the basilar artery, occlusion of an artery of Percheron, cerebral venous sinus thrombosis [CVST]).1 Clinical history, other imaging features, and laboratory analysis narrow the diagnostic possibilities.
丘脑病变的鉴别诊断比较广泛，双侧丘脑MRI异常的病因包括肿瘤性（神经胶质瘤或淋巴瘤）、代谢性和中毒性（Wernicke脑病、渗透性髓鞘溶解、泛酸激酶缺乏、Wilson病、肝脏疾病、缺氧缺血性脑病和一氧化碳中毒）、感染性（西尼罗河病毒或其他虫媒病毒、克-雅氏病及弓形体病）、炎症性（神经白塞病）和血管性（基底动脉尖闭塞，Percheron动脉闭塞和颅内静脉窦血栓形成[CVST]）[1]。 临床病史、影像学特征及实验室检查可缩小可能诊断的范围。
Repeat MRI revealed microhemorrhage, T2 and fluid-attenuated inversion recovery signal abnormality (figure, A), and patchy postcontrast enhancement in the bilateral thalami. Additionally, our patient had already been treated with prednisone in Morocco with subsequent worsening. Based on the bilateral midline signal abnormalities with only patchy enhancement and lack of steroid responsiveness, we suspected a vascular etiology, specifically a straight sinus venous thrombosis, rather than a vasculitic, meningoencephalitic, or neoplastic etiology. Magnetic resonance angiography results were normal; however, magnetic resonance venography showed prominent collateral veins along the left occipital lobe and nonvisualization of the internal cerebral veins, distal vein of Galen, and straight sinus (figure, B).
复查脑MRI示微出血，T2加权和FLAIR序列信号异常（图 A）及双侧丘脑斑片状强化。此外，患者在摩洛哥应用泼尼松治疗后病情出现恶化。根据双侧中线片状强化信号和缺乏类固醇反应，我们怀疑是血管性病变，特别是直窦静脉血栓形成的可能性大，而非血管炎、脑膜脑炎或肿瘤性病变。MRA结果正常；然而，MRV显示沿左侧枕叶走行的侧支静脉，大脑内静脉、Galen静脉远端和直窦不显影（图 B）。 图 栓塞前和栓塞后的神经影像 (A)左侧丘脑FLAIR高信号，右侧明显，伴斑片状强化（未显示），梯度回波序列示微出血（未显示），符合亚急性静脉梗死。(B)箭头所示：闭塞的Galen静脉和直窦。(C)栓塞前DSA：左颈总动脉注射后，图像显示左侧环池早期充盈的曲张静脉（箭头），主要由左侧脑膜中动脉的细小分支供血，符合硬脑膜动静脉瘘（dAVF）。(D)栓塞后DSA图像：dAVF栓塞剂 （onyx）治疗后，没有充盈的血管残留。
Questions for consideration 1. What is the differential diagnosis for CVST? 2. What is the appropriate next step in diagnostic evaluation? 思考问题 1.脑静脉窦血栓形成（CVST）的鉴别诊断是什么？ 2. 下一步如何进行诊断性评估？ CVST is observed most commonly in patients with acquired or inherited hypercoagulability, high estrogen states, cancer, and, uncommonly, head and neck infections.2 In our patient, nonvisualization of the straight sinus was seen along with prominent collateral veins and thalamic ischemia or infarction. These findings suggested a dural arteriovenous fistula (dAVF) and prompted angiography.3Angiography confirmed straight sinus thrombosis and revealed early filling of a venous varix supplied by small branches of the left middle meningeal artery and dural branches of the left posterior cerebral artery,which were consistent with a dAVF (figure, C).
CVST在获得性或遗传性高凝状态、高雌激素状态和癌症患者中最常见，而在头颈部感染患者中少见[2]。本例患者中，直窦不显影,伴有明显的侧支静脉和丘脑缺血/梗死。上述结果提示硬脑膜动静脉瘘（dAVF）[3]，行DSA检查后证实直窦血栓形成，并发现早期充盈的曲张静脉由左侧脑膜中动脉的细小分支和左侧大脑后动脉的硬脑膜分支供应，与dAVF的表现一致（图，C）。 Questions for consideration 1. What interventions are indicated? 2. What is the pathophysiologic relationship among thalamic injury, CVST, and dAVF? 思考问题 1. 需采取什么干预措施？ 2. 丘脑损伤，CVST和dAVF之间的病理生理学关系是什么？ We elected to treat the patient by embolization of the dAVF and with anticoagulation for venous thrombosis. Angiography demonstrated complete obliteration of the dAVF at the end of embolization (figure, D). MRI was repeated 1 month later and showed nearly complete resolution of the thalamic T2 hyperintensities. There was no change in the neurologic examination at that time; however, persistent CVST was seen. The patient was subsequently lost to follow-up.
患者dAVF行栓塞治疗，静脉血栓形成行抗凝治疗。栓塞治疗后行血管造影，显示dAVF完全闭合（图D）。1个月后复查颅脑MRI示丘脑T2高信号基本消失，神经系统查体无变化；然而CVST仍存在。随后该患者失访。 Dural arteriovenous fistulas are rare vascular malformations in which meningeal arteries drain directly into dural venous sinuses, meningeal veins, or subarachnoid veins.3 This type of vascular malformation is associated with various neurologic symptoms and deficits including tinnitus, dementia, seizures, parkinsonism, and cerebellar symptoms.3 When seen with cortical venous drainage, they have a 30% hemorrhage risk.4 A retrospective analysis also found a 4.5% incidence of ischemic stroke in a cohort of 134 patients with dAVF;5 of the 6 patients with strokes had venous infarctions.5 Ischemic stroke was nonsignificantly more common in patients who had CVST in addition to dAVF. Although CVST has been described in the setting of dAVF, there are no known reports of this combination as a cause of bilateral thalamic injury and encephalopathy.
硬脑膜动静脉瘘是罕见的血管畸形，其脑膜中动脉直接进入硬脑膜静脉窦、脑膜静脉或蛛网膜下腔[3]。此类血管畸形可导致各种神经症状和功能缺损，包括耳鸣、痴呆、癫痫发作、帕金森病和小脑症状[3]。当出现皮质静脉引流时，出血风险达30％[4]。回顾性分析134例dAVF患者，发现缺血性卒中的发生率为4.5％；6例中风患者中5例患静脉梗死[5]。同时罹患dAVF和CVST的病人中，缺血性卒中并不常见。尽管dAVF可导致CVST形成，但双侧丘脑损伤和脑病二者同时出现的病例，目前未见报道。
Accumulating evidence suggests that this is a unique vascular mechanism by which thalamic compromise results in subacute encephalopathy or rapidly progressive dementia. Three case reports describe patients with similar presentations who were found to have dAVF with thalamic involvement.6–8 Our patient uniquely presented with concomitant CVST. In contrast to our patient, these 3 patients experienced significant clinical improvement after treatment. Perhaps the persistence of the CVST in our patient was responsible for the lack of improvement.
越来越多的证据表明丘脑损害导致亚急性脑病或快速进展痴呆是一种特殊的血管性机制。3例病例报告描述了具有类似表现的患者，发现dAVF合并丘脑病变[6-8]，3例患者经过治疗后临床症状显著改善；本例患者的特殊之处在于合并CVST，本例患者症状改善不明显，可能与CVST持续存在有关。
The co-occurrence of dAVF and CVST in our patient prompts consideration of the theoretical pathophysiologic relationship between these 2 processes and thalamic injury.5,9 Ischemia associated with a dAVF, for example of the bilateral thalami, may result from the development of venous reflux. Sinus thrombosis results in venous hypertension, which may promote the formation of the dAVF by opening dural arteriovenous shunts and produce ischemia secondary to venous reflux and relative stasis. Alternatively, turbulence in the dAVF may promote thrombus formation.
本例患者dAVF和CVST同时存在，考虑这两个疾病进程和丘脑损伤之间可能存在理论上的病理生理学关系[5，9]。dAVF导致的缺血性事件（例如双侧丘脑）可能由于静脉回流受阻引起。静脉窦内血栓形成导致静脉高压，通过硬脑膜动静脉分流开放、静脉回流受阻和血流瘀滞引发缺血改变，从而促进dAVF的形成；另外，dAVF的湍流也可以导致血栓形成。
With obliteration of the dAVF, flow decreases, venous reflux is eliminated, and perfusion improves. Apparent diffusion coefficient changes (and T2 hyperintensities and contrast enhancement) may then reverse.10 In other words, injury because of edema or ischemia may be reversible. Indeed, such radiographic improvement correlates with clinical improvement in some cases.6–8
随着dAVF消除，血流下降，静脉回流阻断，出现血流灌注改善。然后，ADC的信号改变（T2高信号和对比增强）可以逆转[10]，可能与水肿或缺血引起的损伤可逆性有关。事实上，在某些病例中临床症状恢复与影像学改善有关[6-8]。
Ischemia in the context of a dAVF and CVST is therefore a potentially treatable condition. Appropriate treatment is primarily endovascular embolization of the dAVF, which resulted in reversal of T2 signal abnormalities in our patient, along with anticoagulation for the CVST. Alternative treatments for dAVF include surgical intervention and stereotactic radiosurgery.3
dAVF合并CVST导致的缺血性事件是一种可治性疾病，治疗主要包括：1）dAVF的血管内治疗，栓塞后患者异常的T2信号好转； 2）CVST的抗凝治疗。dAVF的替代治疗包括外科手术治疗和立体定向放射性治疗[3]。
Since thalamic injury attributed to both dAVF and CVST may be reversible, and the 2 processes require distinct treatments, it is imperative to consider CVST with associated dAVF in the differential diagnosis for bilateral thalamic MRI abnormalities.
由于dAVF和CVST导致的丘脑损伤是可逆的，两者需要不同的治疗方案，因此，MRI出现双侧丘脑异常改变，在鉴别诊断中考虑CVST和dAVF是非常必要的。  诗文赏析 Nobody grows old merely by a number of years. We grow old by deserting our ideals. 年轮的累加，并非意味着垂老；丢弃理想，才是真正意义的暮年。 编辑：李会琪 ：李神经群招贤榜--招募病历讨论总结员，公众号编辑的通知【公告】 |
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