Transient perivascular inflammation of the carotid artery syndrome following ischemic stroke

A 51-year-old woman with diabetes, rheumatic heart disease, and atrial fibrillation presented to a hospital with a sudden onset of left lower extremity weakness lasting for 40 minutes. Emergency computed tomography (CT) and magnetic resonance imaging (MRI) of the brain showed cerebral infarction in the right middle cerebral artery territory and hemorrhagic exudate in the right basal ganglia (Figure 1A). The patient’s baseline score on the National Institutes of Health Stroke Scale was 13 points. Thrombolytic therapy with 60 mg of intravenous alteplase was administered immediately. Subsequent magnetic resonance angiography (MRA) of the intracranial carotid vessels showed occlusion at the end of the right internal carotid artery (Figure 1B). Digital subtraction angiography was performed under local anesthesia, and aspiration was repeated twice to remove the embolus. After 2 days of thrombolytic and symptomatic supportive treatment, the patient started to complain of strong right-sided neck pain. Laboratory test results showed elevated levels of C-reactive protein (13.06 mg/l; reference range 0–3 mg/l). Contrast-enhanced MRI and ultrasonography demonstrated marked swelling and thickening of the lateral wall surrounding and compressing the C1 segment of the right internal carotid artery, with low flow velocity and high resistance. A membrane-like structure protruding into the artery lumen about 3.3 cm from the right carotid bifurcation, resulting in severe stenosis, was also visualized (Figure 1C–1F). Finally, transient perivascular inflammation of the carotid artery (TIPIC) syndrome with dissection of the C1 segment of the right carotid artery was diagnosed. Additional anti-inflammatory treatment with methylprednisolone sodium succinate 80 mg once daily for 1 week was administered, with a good clinical and imaging response (Figure 1G). Approximately 3 months later, the patient was admitted again for right-sided carotid stenting (Figure 1H), and subsequently made a full recovery.

TIPIC syndrome, formerly known as idiopathic carotidynia, is a rare vascular disease. Its current name was first used by Lecler et al¹ in 2017. The following features may help diagnose this syndrome: acute pain overlying the carotid artery, which may or may not radiate to the head; eccentric perivascular infiltration (PVI) on imaging; exclusion of another vascular or nonvascular diseases with imaging; and improvement within 14 days, either spontaneous or following anti-inflammatory treatment.¹ Previous studies have reported that PVI in TIPIC syndrome is predominantly eccentric, with only a few cases presenting with mild luminal stenosis and no neurologic symptoms or hemodynamic abnormalities.^1-2 Both PVI enhancement and nonenhancement may present on MRA, CT angiography, or contrast-enhanced ultrasonography,^1-4 but whether these findings are indicative of the disease activity requires further validation, particularly based on pathologic evidence. TIPIC syndrome needs to be differentiated from other large vessel vasculitides, especially Takayasu arteritis (TA). TA predominantly affects young women, and its pathological characteristics include involvement of all arterial layers with various inflammatory infiltrates, including acute exudative, chronic, and granulomatous inflammation, primarily concentrated in the media and adventitia, whereas hyperplasia and neovascularization can be observed in the intimal layer.⁵ In contrast, inflammation in TIPIC syndrome primarily involves the perivascular soft tissue rather than the middle or intimal layer of the artery.

Here, we demonstrated a rare case of TIPIC syndrome with severe carotid artery stenosis and co-occurrence of ischemic stroke (which was caused by carotid artery dissection). Although the underlying pathogenesis of TIPIC syndrome is unclear and its relationship with cerebrovascular events has not been elucidated, ischemic stroke can promote endothelial cell infection and inflammation, and under the condition of oxidative stress, TIPIC syndrome may be induced. However, further research is necessary to confirm this hypothesis.