Both of the patients with AFIB also had ICA stenosis on the ipsilateral side (both measuring 60% according to ECST criteria).

Summarizing, no patient with TA had a visible spot sign. The spot sign was detectable in 10 out of 13 patients (73%) with CRAO. With the exception of one patient, CRAOs were not associated with TA. Taken in account only the patients with embolic CRAO (12 out of 13) the spot sign was present in 83% of the cases. No spot sign could be seen in patients with other forms of ischemic optic neuropathy (e.g. AION, retinal artery branch occlusion). Using the exact Fisher test comparing the frequency of the spot sign in TA and non-TA patients we found a p-value Epacadostat manufacturer of 0.01, the sensitivity of detecting embolic CRAO using the “spot sign” was 83% (95% CI: this website 65–99%). The specificity for embolic occlusions was 100% (95% CI: 65–100%). In this prospective study we demonstrate the diagnostic significance of retrobulbar ultrasonography for the differentiation of embolic and vasculitic causes of ischemic optic neuropathy. The causes for ION can be subdivided into different groups, depending on the affected retinal arteries: CRAO, AION and PION [11]. TA, embolism or hypoperfusion are responsible for retinal ischemia in all subgroups. Reliable techniques to discriminate between the

different forms are funduscopy and fluorescence angiography. Moreover FA can be helpful to show delayed nearly filling or vascular leakage in choroidal vessels in AION for example. However, both methods cannot elicitate the underlying etiology because they lack sensitivity or depth penetration beyond the retina and thus cannot elucidate the underlying cause of ION. Temporal arteritis (Horton disease or giant cell arteritis) and embolism from cerebrovascular disease require different acute and long-term therapeutic managements: for an embolic event, anticoagulation or platelet inhibition plus control of vascular risk factors should be initiated; whereas in TA, rapid initiation and long-lasting steroid therapy is essential. Due to the significant side effects

of long-term steroid treatment, it is clear that a correct diagnosis is mandatory. So far, the only valid list of diagnostic criteria for TA has been established by the American College of Rheumatology. According to the ACR, 3 or more of the following criteria must be present for a diagnosis of TA: (1) age of 50 years or older; (2) new onset of localized headache; (3) temporal artery tenderness on palpation or decreased pulsation; (4) ESR of 50 mm/h or higher; (5) abnormal findings of a temporal artery biopsy. The sensitivity for this diagnosis was reported to be 93.5%, with a specificity of 91.2% for the discrimination of giant cell arteritis from other forms of vasculitis [12]. The main disadvantage of these criteria is that they were not developed and validated for diagnosis in the general population [13].