However, one clinical scenario that could more closely mimic RCVS would be a patient experiencing a small sentinel hemorrhage from a cerebral aneurysm, which could produce a similar clinical course with waxing and waning symptoms.55, Imaging can also help differentiate RCVS from aneurysmal (or perimesencephalic) subarachnoid hemorrhage. Finally, we will discuss how to integrate both clinical and radiographic features in suspected cases of RCVS to formulate a tailored differential diagnosis. Ducros and Bousser2 found that noninvasive imaging with MRA and CTA demonstrated sensitivity for detecting RCVS-vasoconstriction of 80% compared with conventional angiography. Intra-arterial application of nimodipine in reversible cerebral vasoconstriction syndrome: a diagnostic tool in select cases? Newer imaging techniques, including high-resolution vessel wall imaging, may help in the future to better discriminate reversible cerebral vasoconstriction syndrome from primary angiitis of the CNS, an important clinical distinction. 2007 Oct;14(10):1085-7. doi: 10.1111/j.1468-1331.2007.01830.x. However, the validity of this technique remains uncertain. Conversely, hyperacute vasospasm may occasionally be associated with aneurysmal subarachnoid hemorrhage.15 Consequently, considering the patient's overall clinical picture and radiographic features may be the most effective way of differentiating these 2 entities. Headache can remain the sole symptom of RCVS. SUMMARY: The diagnostic evaluation of a patient with reversible cerebral vasoconstriction syndrome integrates clinical, laboratory, and radiologic findings. Although PACNS can produce a pattern of multifocal narrowing and irregularity of mid-to-distal cerebral arteries that is indistinguishable from RCVS, most cases will appear unremarkable on angiographic imaging.2,18⇓–20 This appearance is true even for the criterion standard of conventional angiography, which has a reported sensitivity of only 20%–64% for detecting CNS vasculitis.7,10,29 Alternatively, cerebral vasoconstriction is often apparent in cases of RCVS at presentation or shortly thereafter. Primary angiitis of the central nervous system and reversible cerebral vasoconstriction syndrome. On axial T1 precontrast high-resolution VWI (C), there is intrinsic T1 mural hyperintensity in involved MCA (white arrows) and anterior cerebral artery branches. Axial CBF pulsed arterial spin-labeling maps (A and B) show multiple regions of diminished perfusion involving anterior cerebral artery/MCA watershed territories (black arrows), with T2* DSC perfusion time-to-peak maps (C) demonstrating delayed time-to-peak in these same regions (white arrows). Curr Atheroscler Rep. 2013 Aug;15(8):346. doi: 10.1007/s11883-013-0346-4. Reversible Cerebral Vasoconstriction Syndrome, Part 2: Diagnostic Work-Up, Imaging Evaluation, and Differential Diagnosis, Quantifying Intra-Arterial Verapamil Response as a Diagnostic Tool for Reversible Cerebral Vasoconstriction Syndrome, A unique case of cabergoline-induced reversible cerebral vasoconstriction, OnabotulinumtoxinA injections: treatment of reversible cerebral vasoconstriction syndrome chronic daily headaches, Added Value of Vessel Wall Magnetic Resonance Imaging for Differentiation of Nonocclusive Intracranial Vasculopathies, L37: reversible cerebral vasoconstriction syndrome—distinction from CNS vasculitis, Reversible cerebral vasoconstriction syndrome, Reversible cerebral vasoconstriction syndrome (RCVS) in antiphospholipid antibody syndrome (APLA): the role of centrally acting vasodilators—case series and review of literature, Multimodal imaging of reversible cerebral vasoconstriction syndrome: a series of 6 cases, Reversible cerebral vasoconstriction syndrome: updates and new perspectives, Dramatic intracerebral hemorrhagic presentations of reversible cerebral vasoconstriction syndrome: three cases and a literature review, Narrative review: reversible cerebral vasoconstriction syndromes, Dual energy computed tomography angiography for the rapid diagnosis of reversible cerebral vasoconstriction syndromes: report of a case, Postpartum angiopathy and other cerebral vasoconstriction syndromes, Reversible cerebral vasoconstriction syndromes presenting with subarachnoid hemorrhage: a case series, Call-Fleming syndrome: headache in a 16-year-old girl, The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome: a prospective series of 67 patients, Benign angiopathy of the central nervous system: cohort of 16 patients with clinical course and long-term followup, Primary angiitis of the central nervous system and reversible cerebral vasoconstriction syndrome, Reversible cerebral vasoconstriction syndrome associated with subarachnoid hemorrhage, Reversible cerebral vasoconstriction syndromes: analysis of 139 cases, Pitfalls in the diagnosis of reversible cerebral vasoconstriction syndrome and primary angiitis of the central nervous system, High-resolution MRI vessel wall imaging: spatial and temporal patterns of reversible cerebral vasoconstriction syndrome and central nervous system vasculitis. Five of six patients who underwent lumbar puncture presented with CSF leucocyte levels ≥ 10/mm³. Conventional angiography remains the imaging criterion standard for the evaluation of cerebral vasculature and may detect cerebral vasoconstriction in patients whose initial noninvasive vascular imaging findings appear unremarkable.12 This is particularly true in the evaluation of small, distal cortical vessels, which are suboptimally evaluated by CTA or MRA secondary to their inferior spatial resolution. Most of these patients were diagnosed with cerebral amyloid angiopathy. Primary angiitis of the central nervous system: differential diagnosis and treatment. Enter multiple addresses on separate lines or separate them with commas.  |  In these instances, better visualization of the character and distribution of cerebral artery irregularity and the morphology of any cerebral aneurysms present can be helpful. Postpartum women are one specific subgroup of patients who are at increased risk for both disease entities.1 MR imaging, including susceptibility sequences and MRV, can provide high specificity for the diagnosis of cortical vein thrombosis. 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. 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. However, most patients with a history of migraine who present with RCVS describe the quality and severity of the pain as being different from that in their typical migraine.7,15 Ischemic stroke in patients with migraine tends to be limited to a single vascular territory, as opposed to RCVS, in which multiterritory involvement is common.7. As previously discussed, presenting symptoms, sequelae, and radiographic features of RCVS can significantly overlap other frequently encountered medical conditions involving the CNS (Table 2).1,2,7,15,21,23 Furthermore, treatment of some of these alternative diagnoses, including aneurysmal subarachnoid hemorrhage and PACNS, varies considerably from that of RCVS, making an accurate diagnosis critical to ensuring appropriate patient care.13,19 The following section will highlight important clinical and radiologic findings that can help differentiate some of these entities from RCVS. Despite the established diagnostic criteria, RCVS can manifest with CSF leucocyte levels > 10/mm³. DWI (A) demonstrates an acute infarct involving the right thalamus and posterior limb of the internal capsule (white arrow). 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. The patient was diagnosed with RCVS, with subsequent resolution of cerebral vasoconstriction (D). 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. CSF pleocytosis; RCVS; primary angiitis.


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