Case Presentation: Fibro-muscular dysplasia is a segmental, non-atherosclerotic, non-inflammatory vascular disease of unknown etiology usually found in small and medium-sized arteries. Renal arteries (60–75%) are most commonly involved, followed by the cervico-cranial arteries (25–30%). FMD of cervico-cranial arteries in absence of renal artery involvement is much rarer. We present a case of symptomatic fibro-muscular dysplasia in a young healthy female.

Discussion: This is a case of 49 year old Caucasian female patient with known past medical history of hypothyroidism who presented to the hospital emergency department with sudden onset of visual disturbances, severe headache, numbness and tingling sensation of her left arm. On further questioning, she described her visual disturbances of having black and silver floaters scattered throughout her visual field associated with pulsatile tinnitus and whooshing sensation in her ears. On physical exam, the National Institutes of Health stroke scale (NIHSS) was 0 on admission. CT head without contrast showed no evidence of acute intracranial hemorrhage or infarction. CT angiography showed narrowing of the right V4 segments of the vertebral artery along with beaded appearance of proximal left ICA suspicious for Fibro-muscular Dysplasia with no evidence of aneurysm, carotid or vertebral artery dissection. MRA abdomen was done which showed no involvement of the renal arteries. Neurosurgery evaluated the patient and recommended continuing aspirin, statin and follow-up as outpatient for close monitoring.

Conclusions: Patients with FMD have abnormal cell growth in the walls of their medium–large arteries causing narrowing (stenosis) or enlargement (aneurysm) of these arteries which can have different presentation that vary from being asymptomatic, hypertension (renal artery involvement), or stroke (carotid or vertebral artery involvement).Treatment depends on patient presentation and severity of underlying disease. Patient with asymptomatic carotid or vertebral artery FMD should be treated with aspirin for primary prevention of stroke. Patient with symptomatic presentation such as Amaurosis Fugax, TIA or stroke might need intervention with balloon percutaneous transluminal angioplasty or stent placement. If dissection has already developed, patients need initiation of anticoagulation. Our case stresses the importance of early identification of commonly missed symptoms of headache and tinnitus in FMD patient which if not properly followed can result in acute strokes in otherwise low risk population.

IMAGE 1: CT Angio Head and neck showing alternating, beaded and narrowed appearance of the proximal left ICA.

IMAGE 2: MRA neck w/wo contrast showing Subtle alternating beaded appearance of the proximal left ICA.