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Background: Ischemic events caused by carotid artery stenosis are affected not only by stenosis but also by the instability of plaque. In symptomatic low-grade stenosis cases medical treatment is generally performed, but there are cases with repeated recurrence. The purpose of this study is to clarify the clinical features and long-term outcomes in cases of symptomatic low-grade stenosis.Methods: We included seventy-one symptomatic patients with carotid low-grade stenosis (Results: The mean stenosis rate, rSI and ERR on admission were 20.4, 1.75 and 1.96, respectively. Seventy percent of cases involved intraplaque hemorrhage and positive remodeling. During a mean of 52-months follow-up, a recurrence of ischemic events was confirmed in 33 cases (46%), from which the duration until recurrence was within 7 days (33%), 3 months (18%), 1 year (18%), 2 years (21%), and over 2 years (15%). Nine cases had impending stroke, 3 of which were associated with major artery occlusion. CEA was performed in 28 cases (39%) for impending stroke (25%), recurrence of ischemic events (46%), asymptomatic infarction (7%), and stenosis rate progression (11%). During a mean of 47-months follow-up after CEA, 2 cases of death (fetal intracerebral hemorrhage, asphyxia) and one case of brain stem lacunar infarction were observed, but an ipsilateral ischemic event was not observed.Conclusion: Most of the symptomatic patients with low-grade stenosis had both intraplaque hemorrhage and positive remodeling. The risk of recurrence and stenosis progression was high. CEA might have had a preventive effect against ischemic events in low-grade stenosis.