Clinical characteristics of dural arteriovenous fistula presenting primarily as epilepsy: Analysis of 4 cases

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

Abstract

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

CLC Number:

R741

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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URL: https://www.lchc.cn/EN/10.3969/j.issn.1004-583X.2026.07.007

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

Figures/Tables 6

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

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).

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).

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).

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).

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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