Idiopathic extracranial internal carotid artery vasospasm: case report and systematic review

Idiopathic extracranial internal carotid artery vasospasm (IEICAV) is characterized by spontaneous, recurrent, and reversible vasoconstriction of the cervical internal carotid artery (ICA). The etiology remains elusive, and no effective treatment has been established. The present study presents a case of recurrent IEICAV with migraine-like symptoms and conduct a systematic review on IEICAV.

A retrospective analysis was conducted on a case involving medical history, radiological data, treatment, and outcomes. A systematic review of published IEICAV cases was conducted through database searching in PubMed, Embase, and Web of Science from inception until May 2024.

A 22-year-old female with recurrent headaches, blurred vision, and aphasia was diagnosed with bilateral IEICAV through angiography. Magnetic resonance imaging demonstrated a novel cerebral infarction during a prolonged episode. Treatment with topiramate successfully controlled recurrence in a 5-month follow-up. The systematic review included 36 IEICAV cases reported by literature. Bilateral involvement of extracranial ICAs was observed in 25 (69.4%) cases. Cerebral infarction was identified in 31 (88.9%) cases. Despite various treatment attempts including vasodilators, antiplatelet, anticoagulants, glucocorticoids, and other medical or surgical intervention, the recurrent rate increased in 5 (13.9%) cases, decreased in 10 (27.8%) cases, and remained unchanged in 4 (11.1%) cases.

The elusive mechanism of IEICAV brings great difficulty into managing recurrence. Preventing IEICAV-related infarction related to secondary factors like hypoperfusion may be crucial for maintaining life quality. Further research is essential for advancing treatment strategies and a case-by-case approach is needed in identifying and eliminating possible triggers for vasospastic episodes.