It is the lack of specific symptoms and their potential to appear anywhere that makes<unk>  a challenge to detect early on. The most accurate diagnosis comes from combining clinical presentation and angiographic imaging. According to the Michigan Cardiovascular Outcomes Research and Reporting Program (MCORRP, 2013) the length of time from a patient’s first signs or symptoms to diagnosis is commonly 5 years.<unk>  is currently diagnosed through the use of both invasive and non-invasive tests. Non-invasive testing includes duplex ultrasonography, magnetic resonance angiography (MRA), and computed tomography angiography (CTA). Invasive testing through angiography is the gold standard. However, due to the higher risk of complications, this is typically not done early on. Occasionally,<unk>  is diagnosed asymptomatically after an unrelated x-ray presents the classic ‘string of beads’ appearance of the arteries, or when a practitioner investigates an unexpected bruit found during an exam. When a diagnosis of<unk>  is considered for a patient thorough medical history, family history, as well as vascular examination, should be completed. A definitive diagnosis of<unk>  can only be made with imaging studies. Catheter-based angiography (with contrast) has proven to be the most accurate imaging technique: this test involves a catheter is inserted into a large artery and advanced until it reaches the vessel of question. The catheter allows practitioners to view and measure the pressure of the artery aiding in the categorization and severity of the<unk>  diseased artery. According to Olin, “catheter-based angiography is the only imaging modality that can accurately identify the changes of<unk> , aneurysm formation, and dissection in the branch vessels.” Practitioners believe it is important to utilize IVUS imaging because stenosis can sometimes only be detected through the methods of pressure gradient or IVUS imaging. In addition, computed tomography angiography and magnetic resonance angiography are commonly used to evaluate arteries in the brain. Doppler ultrasound may be used in both the diagnosis and follow-up of<unk> . Similar diseases * In the visceral distribution, Segmental arterial mediolysis may mimic<unk> . * In the visceral and cerebrovascular distribution, atherosclerosis must be considered. Children The differentiating presentations are suggestive of<unk>  being a unique syndrome in respect to the pediatric population. Experienced<unk>  clinicians warn against relying on the “string of beads” angiography for a diagnosis. In fact, it is suggested that<unk>  may be both under and over-diagnosed in children with stroke.
Fibromuscular dysplasia
Arteriosclerosis obliterans
Familial myxovascular fibromas
Endocardial fibroelastosis
Fibromatosis
Familial thoracic aortic aneurysm
Actinic elastosis
Collagen disease