Indicates open access to non-subscribers at www.ajnr.org. Most of these patients were diagnosed with cerebral amyloid angiopathy. These results suggest that VWI may be a useful tool in differentiating RCVS and CNS vasculitis, though further investigation is needed (Fig 2). 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. On axial T1 precontrast high-resolution VWI (C), there is intrinsic T1 mural hyperintensity in involved MCA (white arrows) and anterior cerebral artery branches. Ducros and Bousser2 found that noninvasive imaging with MRA and CTA demonstrated sensitivity for detecting RCVS-vasoconstriction of 80% compared with conventional angiography. 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. The resulting patient radiation exposure is a potential drawback of this method, particularly in those patients requiring multiple scans. How often is thunderclap headache caused by the reversible cerebral vasoconstriction syndrome? A 35-year-old man with a history of Behçet vasculitis who presented with left-sided weakness. In most cases of RCVS, findings of CSF analysis will be unremarkable, with red and white blood cell counts and protein levels either within normal limits or only mildly elevated.2,7,9⇓⇓⇓–13 Finally, findings of other laboratory tests, including serum analysis for markers of inflammation such as erythrocyte sedimentation rate and C-reactive protein, are also usually within normal limits in patients with RCVS.2,9,14, The role of neuroimaging in patients with RCVS includes demonstration of cerebral vasoconstriction, evaluation of alternative diagnoses, and monitoring potential complications such as intracranial hemorrhage, vasogenic edema, and ischemic stroke.4,7,9 Although conventional angiography has been the criterion standard for evaluation of cerebral vasoconstriction in suspected cases of RCVS, noninvasive imaging modalities such as transcranial Doppler sonography (TCD), CT angiography, and MR angiography are being used with increasing frequency (Table 1).4,5,9,12,15 When present, cerebral vasoconstriction involves multiple vascular territories and results in a beaded appearance of medium-to-large cerebral arteries with multifocal areas of narrowing interspersed with normal-caliber segments.1,4,7,12,14 The severity and distribution of vasoconstriction can fluctuate among examinations, with some areas improving and others worsening.1,2,9,16,17 Although the above angiographic findings are highly suggestive of RCVS in the appropriate clinical setting, they remain nonspecific and can be encountered with various other types of CNS vasculopathies and vasculitis.2,14,18⇓–20, Role of imaging modalities in the management of RCVS, The initial angiographic evaluation findings in suspected cases of RCVS may be unremarkable in the 4–5 days following patient presentation.1,2,4,5,21 In fact, cerebral vasoconstriction may not be visualized in up to one-third of patients with RCVS during the first week following symptom onset.22 As suggested by Ducros and Bousser,2 this finding may be due to segmental vasoconstriction in RCVS beginning in small, peripheral arterioles before subsequently proceeding centripetally to involve medium and large cerebral arteries, which are more readily visualized.23 If cerebral vasoconstriction is not demonstrated on initial vascular imaging and other diagnoses have been excluded, the patient should be managed as if he or she has possible or probable RCVS.1. Distinguishing RCVS from primary angiitis of the central nervous system (PACNS) is essential to avoid unnecessary and sometimes unfavourable immunosuppressive treatment. Five of six patients who underwent lumbar puncture presented with CSF leucocyte levels ≥ 10/mm³. No patient had further cerebral strokes or bleedings without immunosuppressive treatment over more than 12 weeks. 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.
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