String-of-beads at First Sight: Cerebrovascular Fibromuscular Dysplasia Presenting as an Acute Ischemic Stroke
Rula Saeed1, Shikhar Khurana1, Maryama Mohamed, MD1, Davis Pathadan1, Jonathan Dissin1
1Neurology, Jefferson Einstein Philadelphia Hospital
Objective:
To report a case of bilateral cervical ICA FMD with an extracranial ICA pseudoaneurysm and a synchronous intracranial PCoA aneurysm presenting as an acute stroke, and to detail the decision to proceed with IV thrombolysis after vascular lesions were identified.
Background:
Fibromuscular dysplasia (FMD) is a non-atherosclerotic arteriopathy of medium-sized arteries classically involving the carotid and renal beds. Cerebrovascular FMD may present with ischemic symptoms, aneurysms, or dissection; concurrent extracranial pseudoaneurysm with synchronous intracranial aneurysm at index presentation is unusual.
Design/Methods:
Not applicable
Results:
A 41-year-old woman with hypertension presented with sudden facial droop, right arm weakness and numbness, and blurred vision with NIHSS 4. Non-contrast CT was normal. CTA showed bilateral mid-to-distal cervical ICA “string-of-beads” appearance compatible with FMD and a 2.5-mm saccular pseudoaneurysm of the left mid-cervical ICA. A 1.5-mm saccular aneurysm at the right PCoA was also noted. With disabling dominant-upper-limb deficits and no contraindications, IV thrombolysis was administered. Symptoms improved rapidly; post-tPA imaging showed no hemorrhage, and MRI revealed no acute infarct. Duplex renal ultrasound showed no evidence of renal FMD. She was discharged neurologically intact on antiplatelet therapy, with a plan for 6–12-month CTA/MRA surveillance and comprehensive vascular screening.
Conclusions:
This case underscores that cerebrovascular FMD can present with simultaneous extracranial pseudoaneurysm and intracranial aneurysm and still warrant timely IV thrombolysis for disabling deficits after careful risk assessment. We demonstrate that timely IV thrombolysis can be appropriate and safe in the presence of a small, unruptured intracranial aneurysm, consistent with observational data and guideline thresholds that flag aneurysms ≥10 mm as higher risk. Because cerebrovascular FMD frequently coexists with or predisposes to cervical artery dissection, contemporary guidance supports IVT in otherwise-eligible dissection patients within 4.5 h, reinforcing that FMD itself is not a contraindication. 
10.1212/WNL.0000000000217754
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