以癫痫为主要表现的硬脑膜动静脉瘘4例临床特征分析

doi:10.3969/j.issn.1004-583X.2026.07.007

摘要/Abstract

摘要：

目的分析以癫痫为主要表现的硬脑膜动静脉瘘(dural arteriovenous fistula, DAVF)临床特征、误诊原因及癫痫发作机制,提升临床对该疾病的认识及诊断准确率。方法回顾性分析4例以癫痫发作为主要表现DAVF患者的临床资料,并结合相关文献总结。结果本组4例均为男性,平均发病年龄53.5岁,核心临床表现均为全面性癫痫发作。3例早期误诊为脑梗死、帕金森综合征。误诊原因包括首发症状不典型、未及时完善脑血管评估、对静脉窦病变致癫痫认识不足。最终均经脑血管造影确诊为乙状窦DAVF,3例合并同侧乙状窦狭窄/闭塞。治疗方案,3例行经动脉Onyx胶栓塞术+乙状窦球囊扩张+支架植入术,1例行单纯经动脉Onyx胶栓塞术。术后随访显示,2例癫痫发作控制,预后良好。1例术后继发脑积水,外科手术后脑出血并遗留痴呆。1例病程长达10年,进展为难治性癫痫持续状态,放弃治疗后死亡。结论以癫痫起病的DAVF极易误诊。静脉窦回流受阻、皮层静脉高压、血脑屏障破坏是诱发癫痫的核心机制,同侧的乙状窦狭窄进一步加重血流动力学紊乱,促进癫痫进展。DAVF是成人晚发性癫痫少见但可根治的病因,对不明原因反复癫痫发作的患者需完善脑电图与脑血管造影,尽早介入干预可改善远期预后。

关键词: 中枢神经系统血管畸形, 癫痫, 误诊

Abstract:

Objective To analyze the clinical characteristics, causes of misdiagnosis, and seizure mechanisms of dural arteriovenous fistula (DAVF) presenting primarily as epilepsy, and to improve clinical awareness and diagnostic accuracy. Methods The clinical data of 4 patients with DAVF presenting mainly with epileptic seizures were retrospectively reviewed, and relevant literature was summarized. Results All 4 patients were male, with a mean age at onset of 53.5 years, and the core clinical manifestation in all cases was generalized epileptic seizures. Three cases were initially misdiagnosed as cerebral infarction and Parkinson syndrome. The reasons for misdiagnosis included atypical initial symptoms, failure to promptly complete cerebrovascular evaluation, and insufficient recognition of seizures caused by venous sinus lesions. All cases were ultimately confirmed by digital subtraction angiography (DSA) as sigmoid sinus DAVF, and 3 cases had concomitant ipsilateral sigmoid sinus stenosis/occlusion. In terms of treatment, 3 patients underwent transarterial Onyx embolization plus sigmoid sinus balloon angioplasty and stent implantation, while 1 patient underwent transarterial Onyx embolization alone. Follow-up after surgery showed seizure control and favorable prognosis in 2 cases. One patient developed secondary hydrocephalus after surgery and later suffered cerebral hemorrhage after surgical treatment, resulting in dementia. One patient had a disease course of up to 10 years, progressed to refractory status epilepticus, and died after discontinuing treatment. Conclusion DAVF presenting with epilepsy is highly prone to misdiagnosis. Impaired venous sinus drainage, cortical venous hypertension, and blood-brain barrier disruption are key mechanisms triggering seizures. Ipsilateral sigmoid sinus stenosis further exacerbates hemodynamic disturbance and promotes seizure progression. DAVF is a rare but curable cause of late-onset epilepsy in adults. For patients with recurrent seizures of unknown cause, electroencephalography and DSA should be performed promptly, and early interventional treatment may improve long-term prognosis.

Key words: central nervous system vascular malformations, epilepsy, diagnostic errors

中图分类号:

R741

董思语, 高甜文, 孙美, 金善泉. 以癫痫为主要表现的硬脑膜动静脉瘘4例临床特征分析[J]. 临床荟萃, 2026, 41(7): 619-625.

Dong Siyu, Gao Tianwen, Sun Mei, Jin Shanquan. Clinical characteristics of dural arteriovenous fistula presenting primarily as epilepsy: Analysis of 4 cases[J]. Clinical Focus, 2026, 41(7): 619-625.

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链接本文: https://www.lchc.cn/CN/10.3969/j.issn.1004-583X.2026.07.007

https://www.lchc.cn/CN/Y2026/V41/I7/619

图/表 6

表1 4例患者的临床资料,治疗及预后

Tab.1 Clinical data, treatment, and prognosis of the 4 patients

序号	性别	年龄	症状	既往史	头颅MRI	脑血管造影	治疗	预后
1	男	57岁	视力下降,癫痫,帕金森	糖尿病、高血压病	右侧颞枕叶异常信号,血管源性水肿	右侧乙状窦动静脉瘘,右侧乙状窦重度狭窄,左侧乙状窦狭窄	经动脉Onyx胶栓塞术+乙状窦球囊扩张+支架植入术	预后良好
2	男	50岁	癫痫,认知障碍	脑梗死	左侧大脑中动脉供血区大片异常信号,考虑脑梗死左颞枕叶扩张,静脉迂曲影	左侧乙状窦动静脉瘘	经动脉Onyx胶栓塞术	预后良好,偶发癫痫
3	男	52岁	癫痫,帕金森	脑外伤,颅内血肿清除术	双侧颞枕叶侧脑室旁异常信号,静脉梗死可能	左侧乙状窦动静脉瘘,左侧乙状窦闭塞,右侧乙状窦狭窄	经动脉Onyx胶栓塞术+乙状窦球囊扩张+支架植入术	癫痫持续状态,放弃治疗后死亡
4	男	65岁	癫痫,认知障碍	糖尿病、高血压病	左侧额顶枕叶梗死灶伴出血转化	左侧窦汇区动静脉瘘,左侧乙状窦交汇处闭塞,左侧乙状窦狭窄	经动脉Onyx胶栓塞术+乙状窦球囊扩张+支架植入术	术后脑积水,侧脑室腹腔分流术后脑出血,痴呆状态

图1 病例1 a~c.头颅MRI Flair、DWI、ADCs,示右侧颞枕叶异常信号,考虑血管源性水肿可能;d.右侧乙状窦DAVF,动脉早期即显影的异常血管团,瘘口区域向乙状窦引流(箭头所示)。

Fig.1 Case 1 a-c. Brain MRI Flair, DWI, and ADCs show abnormal signal in the right temporo-occipital lobe, suggesting the possibility of vasogenic edema; d. Right sigmoid sinus DAVF: an abnormal vascular mass is visualized in the early arterial phase, with drainage from the fistula area into the sigmoid sinus (as indicated by the arrow).

图2 病例2 a~c.头颅MRI Flair、DWI、ADCs示左侧颞叶脑梗;d~e.CTA显示左侧颞枕叶增粗迂曲静脉血管影;f.异常扩张的颈外动脉分支,颅底硬脑膜区域斑片状异常强化影,对应DAVF瘘口(箭头所示)。

Fig.2 Case 2 a-c. Brain MRI Flair, DWI, and ADCs show cerebral infarction in the left temporal lobe; d-e. CTA shows thickened and tortuous venous vascular shadows in the left temporo-occipital lobe; f. Abnormally dilated branches of the external carotid artery and patchy abnormal enhancement in the skull base dural region, corresponding to the DAVF fistula site (as indicated by the arrow).

图3 病例3 a.头颅CT示右侧额叶出血,左侧颞叶见斑片状低密度影;b~c.DSA示左侧乙状窦DAVF,左侧乙状窦闭塞,右侧乙状窦近心端狭窄明显;d.示异常血流经左侧岩窦逆流至左侧海绵窦(箭头所示)。

Fig.3 Case 3 a. Brain CT shows hemorrhage in the right frontal lobe and patchy low-density areas in the left temporal lobe; b-c. DSA shows left sigmoid sinus DAVF, left sigmoid sinus occlusion, and marked stenosis at the proximal segment of the right sigmoid sinus; d. Abnormal blood flow refluxes through the left petrosal sinus into the left cavernous sinus (as indicated by the arrow).

图4 病例4 a~c.头颅MRI Flair、DWI、ADC示左侧额颞顶叶亚急性梗塞灶伴出血转化; d.左侧侧窦区及窦汇区DAVF(箭头所示)

Fig.4 Case 4 a-c. Brain MRI Flair, DWI, and ADC show a subacute infarct in the left frontotemporoparietal lobe with hemorrhagic transformation; d. DAVF in the left transverse sinus and torcular region (as indicated by the arrow).

图5 长程视频EEG a.清醒期示左侧枕区可见周期性单侧癫痫样放电,中央、顶区尖波/尖慢综合波发放;b.思睡期示双侧额、中央、顶、枕区非同步散在尖波

Fig.5 Long-range video EEG a. During wakefulness, periodic unilateral epileptiform discharges are seen in the left occipital region, with spike/sharp-and-slow-wave discharges in the central and parietal regions; b. During drowsiness, asynchronous scattered spikes are seen in the bilateral frontal, central, parietal, and occipital regions.

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