As is the case with primary angiitis of the CNS, distinguishing RCVS from cortical vein thrombosis is critical because treatment of the latter often entails anticoagulation, which carries significant risks and has not been shown to be beneficial in RCVS. RCVS generally follows a benign, self-limited course with supportive care, while clinical deterioration would be expected in PACNS without prompt immunosuppressive therapy.2,18, Nonvascular imaging findings can also help differentiate PACNS and RCVS. In patients younger than 60 years of age, presentation with abrupt, severe headache was common, and most of these individuals were presumptively diagnosed with RCVS. Finally, we will discuss how to integrate both clinical and radiographic features in suspected cases of RCVS to formulate a tailored differential diagnosis. Hyperintense vessels along cerebral sulci on T2 FLAIR imaging have been noted in patients with RCVS (22%) and correlate with more severe vasoconstriction as measured by TCD.16,26⇓–28 In one study, the presence of hyperintense vessels was associated with a higher risk incidence of ischemic stroke and posterior reversible encephalopathy syndrome.26 Hyperintense vessels on T2 FLAIR imaging have previously been described in association with other conditions involving severe cerebral artery stenosis or occlusion, including acute large-vessel ischemic stroke and Moyamoya disease. Intracranial vasospasms (a) in the right middle cerebral artery (MCA) resolving by intravenous nimodipine infusion over 20 min (b, c). Cortical vein thrombosis is another potential cause of both thunderclap headache and convexity subarachnoid hemorrhage and should be considered in the differential diagnosis with RCVS in the appropriate clinical setting. 2010 Jun;24(3):413-26. doi: 10.1016/j.berh.2009.12.003. Ischemic infarctions in RCVS are typically watershed in location and bilateral, presumably reflecting impaired cerebral blood flow secondary to severe cerebral vasoconstriction.2,7 In addition, MR imaging can also evaluate potential alternative diagnoses, including PACNS, dural sinus thrombosis, pituitary apoplexy, cortical vein thrombosis, and arterial dissection. Description: Headache caused by reversible cerebral vasoconstriction syndrome, typically thunderclap headache recurring over 1-2 weeks, often triggered by sexual activity, exertion, Valsalva manœuvres and/or emotion. Additionally, DSA may provide complementary information to aid the diagnosis, including reversibility of vasoconstriction following intra-arterial administration of a vasodilator.48⇓⇓⇓–52 Because diagnostic confirmation of RCVS is usually retrospective following spontaneous resolution of clinical and angiographic findings in 1–3 months, there is often a substantial delay in confirming the diagnosis. Distinguishing RCVS from primary angiitis of the central nervous system (PACNS) is essential to avoid unnecessary and sometimes unfavourable immunosuppressive treatment. However, because reversibility of the cerebrovascular angiographic abnormalities is necessary for the diagnosis, it would appear that many, if not most, patients have a full recovery without residual symptoms. RCVS, cocaine vasculopathy demonstrates arterial wall in-flammation on histopathologic evaluation,11 Two of the patients with persistent arterial narrowing were included in previous studies4,11 of vessel wall MRI. However, newer imaging techniques, particularly vessel wall imaging, may offer increased specificity for the diagnosis. Subsequent catheter angiograms (A and B) demonstrate marked irregularity of branches of the distal right anterior cerebral artery (white arrow, A) and left MCA (white arrows, B), with multifocal areas of narrowing and saccular and fusiform dilation. Finally, improvement in cerebral artery narrowing following intra-arterial vasodilator therapy has also been proposed as a feature distinguishing RCVS from PACNS.52. The resulting patient radiation exposure is a potential drawback of this method, particularly in those patients requiring multiple scans. Unfortunately, none of these diagnostic criteria, either alone or in combination, are entirely specific for RCVS vasoconstriction or arterial vasospasm. Subarachnoid hemorrhage associated with RCVS is often small in amount and confined to cerebral sulci near the vertex. Despite the established diagnostic criteria, RCVS can manifest with CSF leucocyte levels > 10/mm³. NIH Brain MR imaging is often performed in suspected cases of RCVS, and findings can appear normal or demonstrate evidence of complications of the syndrome, such as watershed infarcts or posterior reversible encephalopathy syndrome.10,12,14,16 For example, T2 FLAIR-weighted imaging can be used to evaluate for subarachnoid hemorrhage and cerebral edema, while diffusion-weighted imaging is helpful in evaluating for watershed infarcts. MR imaging can demonstrate characteristic susceptibility artifacts associated with a superficial cortical vein consistent with thrombus. COVID-19 is an emerging, rapidly evolving situation. Dual-energy CTA may aid in the diagnosis of cerebral vasoconstriction in suspected cases of RCVS and the evaluation of potential alternative diagnoses such as cerebral aneurysm, by improved bone removal at the skull base.8 However, one important drawback of this technique is the increased radiation exposure to the patient.8 Finally, CT venography can also be performed with CTA with a slightly delayed scan following contrast administration, potentially allowing the diagnosis of cortical vein and/or dural sinus thrombosis.
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