Churg-Strauss syndrome (CSS) is known to mainly involve small vessels; thus, in the event of large-vessel disease, other causes must be taken into consideration. We report a case of CSS with recurrent arterial dissection of the cervicocephalic arteries. A 74-year-old man with CSS visited the emergency department because of sudden left hemiparesis. He had been taking corticosteroids as CSS treatment for 12 years. He had a confirmed right vertebral dissection 10 years before and a right distal internal carotid artery dissection 1 year before. As the arterial dissection occurred in the same vessel region a year before, a stent was deployed in the region. A large cervicocephalic artery dissection in patients with CSS that is well regulated with immunotherapy is likely caused by the long-term corticosteroid therapy weakening the vessel wall; therefore, replacement with the appropriate immunotherapy should be considered.
Churg-Strauss syndrome (CSS) is a disseminated small-vessel systemic necrotizing vasculitis with extravascular granulomas that occurs exclusively among patients with asthma and tissue eosinophilia [
Herein, we report a case of CSS with recurrent arterial dissection of the large cervicocephalic arteries and discuss the possible mechanisms of stroke in patients with CSS during long-term steroid therapy.
A 74-year-old man was admitted with sudden left hemiparesis and left-side neglect. He had a history of asthma, hypereosinophilia, mononeuritis multiplex, and paranasal sinus abnormality and was diagnosed as having CSS in accordance with the diagnostic criteria of the American College of Rheumatology [
The patient had multiple episodes of ischemic cerebrovascular events. He had a history of multiple cerebellar infarctions due to the right vertebral artery dissection 10 years before (
Neurological examination revealed a dense left hemiparesis, right-gaze preponderance, left-sided neglect, and dysarthria. His initial National Institutes of Health Stroke Scale (NIHSS) score was 13. The results of the routine laboratory tests, including complete blood count with high-sensitivity C-reactive protein level, chemistry, coagulation test, urine analysis, and chest radiography, were all within the normal limits. Cardiac investigations, including 72-hour telemonitoring and transthoracic echocardiography, revealed negative results.
Brain magnetic resonance imaging revealed an infarction in the territory of the right middle cerebral artery, and MRA revealed an occlusion of the right distal ICA, which increased the suspicion of dissection recurrence (
Written informed consent was obtained from the patient for the publication of this case report.
Ischemic stroke is uncommon in patients with CSS [
The association of spontaneous dissection with CSS or long-term steroid therapy is largely unknown. The advent of corticosteroid treatment undoubtedly improves the overall prognosis of patients with CSS but may have deleterious effects on the cardiovascular system [
Vessel fragility is highly likely related to the long-term corticosteroid therapy in our patient, who experienced frequent (three recurrences) spontaneous cervicocephalic dissections during long-term corticosteroid therapy, with well-controlled CSS. In this case, we decided to switch from the corticosteroid therapy to immunotherapy. Our present case suggests that if spontaneous dissection occurs repeatedly in a patient with an autoimmune disease such as CSS, vessel fragility related to corticosteroid therapy should be suspected and the corticosteroid dose should be decreased or replaced with other immunotherapies. On the contrary, increased corticosteroid or immunotherapy dose could be considered when stroke occurs during deterioration of autoimmune disease.
No potential conflict of interest relevant to this article was reported.
This study was supported by the National Research Foundation of Korea (NRF) grant (No. 2018R1A2B2003489).
Previous brain magnetic resonance images and magnetic resonance angiogram of brain arteries. Occlusion of the right distal vertebral artery (A: arrow) and dark intensity in the right distal vertebral artery, which represents an intramural hematoma (B: arrow). (C) Diffusion-weighted magnetic resonance image of multiple cerebellar acute infarcts affecting both cerebellar hemispheres. (D, E) Right distal internal carotid artery dissection 1 year before (D: arrowheads), showing full recovery on the magnetic resonance angiogram obtained 6 months before (E: arrowheads).
(A) Brain magnetic resonance images of multiple infarctions in the territory of the right middle cerebral artery, and (B) increased time to peak in the corresponding region (perfusion map). (C) Magnetic resonance angiogram showing a tapered occlusion of the right proximal internal carotid artery (ICA) (arrowheads) and abrupt cut-off of the distal ICA (arrow), suggestive of distal ICA occlusion. suggestive of distal ICA occlusion. (D) Catheter angiogram showing a tapered occlusion of the right proximal ICA from the mid cervical region to the petrous segment (arrowheads). (E) By using a Prowler 14 microcatheter (Cordis Corp.), we entered the ophthalmic segment of the distal ICA. (F) Deployment of the carotid wall stent 9/50 (Boston Scientific Corp.) from the distal common carotid artery to the cervical ICA (arrowheads). (G) Deployment of two Enterprise 4/39 stents (Codman neurovascular), one in the petrous segment and the other from the distal petrous segment to the ophthalmic segment (arrows).