Background and purposeIdiopathic intracranial hypertension (IIH) is characterized by abnormally elevated intracranial pressure (ICP) without identifiable etiology. Recently, however, a subset of patients with presumed IIH have been found with isolated internal jugular vein (IJV) stenosis in the absence of intracranial abnormalities. MethodsFifteen consecutive patients were screened from 46 patients suspected as IIH and were finally confirmed as isolated IJV stenosis. The stenotic IJV was corrected with stenting when the trans-stenotic mean pressure gradient (MPG) was equal to or higher than 5.44 cmH(2)O. Dynamic magnetic resonance venography, computed tomographic venography and digital subtraction angiography of the IJV, MPG, ICP, Headache Impact Test 6 and the Frisen papilledema grade score before and after stenting were compared. ResultsAll the stenotic IJVs were corrected by stenting. MPG decreased and the abnormal collateral veins disappeared or shrank immediately. Headache, tinnitus, papilledema and ICP were significantly ameliorated at 14 3 days of follow-up (all P < 0.01). At 12 +/- 5.6 months of outpatient follow-up, headache disappeared in 14 out of 15 patients (93.3%), visual impairments were recovered in 10 of 12 patients (83.3%) and tinnitus resolved in 10 out of 11 patients (90.9%). In 12 out of 15 cases, the Frisen papilledema grade scores declined to 1 (0-2). The stented IJVs in all 15 patients kept to sufficient blood flows on computed tomographic venography follow-up without stenting-related adverse events. ConclusionsNon-thrombotic IJV stenosis may be a potential etiology of IIH. Stenting seems to be a promising option to address the issue of intracranial hypertension from the etiological level, particularly after medical treatment failure.