VWI can be technically challenging to perform however, due to the small caliber and tortuous course of the intracranial arteries, necessitating high spatial resolution and thus high-field-strength magnets. Find NCBI SARS-CoV-2 literature, sequence, and clinical content: https://www.ncbi.nlm.nih.gov/sars-cov-2/. Appropriate care of patients suspected of having RCVS begins with a prompt diagnostic work-up to exclude alternative diagnoses, such as aneurysmal subarachnoid hemorrhage and PACNS.6 This includes obtaining a complete medical history, with particular attention paid to any common RCVS triggers that may be present; detailed physical examination; CSF analysis; and noncontrast head CT to evaluate for intracranial hemorrhage.2,7⇓–9 CSF analysis is an important element in the diagnostic work-up of RCVS, which is reflected in its inclusion in the diagnostic criteria of the syndrome. 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. DWI (A) shows multifocal infarcts involving the centrum semiovale and left posterior parietal lobe. Diagnostic criteria: A. DWI (A) demonstrates an acute infarct involving the right thalamus and posterior limb of the internal capsule (white arrow). Proposed criteria for differentiating RCVS vasoconstriction from SAH vasospasm10. We do not capture any email address. 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. The headache associated with PACNS is often slowly progressive with an insidious onset, differing markedly from the typical thunderclap headache of RCVS in both time course and peak severity.1,2,7,13,16,18,19,57 Patient demographics in these disease entities also demonstrate significant differences. 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. Intracranial vasospasms (a) in the right middle cerebral artery (MCA) resolving by intravenous nimodipine infusion over 20 min (b, c). 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. For example, although RCVS vasoconstriction is often noted to involve distal cerebral arteries, more proximal vessel involvement occurs. MR imaging can demonstrate characteristic susceptibility artifacts associated with a superficial cortical vein consistent with thrombus. 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. Get the latest public health information from CDC: https://www.coronavirus.gov. Differential diagnosis. NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. Advances in primary angiitis of the central nervous system. Although conventional angiography is generally a safe procedure when performed by experienced operators, a suggestion has been made that there may be an increased risk of transient ischemic attack in patients with RCVS. Careful anamnesis and the response of 'vasculitis-like angiography' to nimodipine given as a test during angiography and as oral medication are key to differentiate RCVS from cerebral vasculitis. Finally, improvement in cerebral artery narrowing following intra-arterial vasodilator therapy has also been proposed as a feature distinguishing RCVS from PACNS.52. 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. Clinical and imaging features of RCVS can overlap other disorders of the central nervous system considerably, particularly primary angiitis of the CNS. USA.gov. Distinguishing RCVS from primary angiitis of the central nervous system (PACNS) is essential to avoid unnecessary and sometimes unfavourable immunosuppressive treatment. Headache can remain the sole symptom of RCVS. Diagnostic criteria include normal or near-normal findings in cerebrospinal fluid (CSF) analysis, especially leucocyte levels < 10/mm³. Special emphasis will be placed on differentiating RCVS from aneurysmal subarachnoid hemorrhage and primary angiitis of the CNS (PACNS). This includes high-resolution MR vessel wall imaging (VWI), a new technique that may help differentiate RCVS from alternative diagnoses by characterizing pathologic changes in the wall of affected cerebral arteries. Unfortunately, none of these diagnostic criteria, either alone or in combination, are entirely specific for RCVS vasoconstriction or arterial vasospasm. Finally, we will discuss how to integrate both clinical and radiographic features in suspected cases of RCVS to formulate a tailored differential diagnosis. However, the evolution of cerebral vasoconstriction was not uniform, with some cerebral arteries improving on serial scans and others worsening.29 They also found that combined segmental vasoconstriction scores in the M1 and P2 arterial segments were most closely associated with the complications of ischemic stroke and posterior reversible encephalopathy syndrome.29 One limitation of MRA is the evaluation of small, distal cerebral arteries, which are better evaluated on conventional angiography, given its superior spatial resolution.29, High-resolution MR VWI is a relatively new technique that is being increasingly used in the evaluation of cerebrovascular disease, including CNS vasculitis, RCVS, cerebral aneurysms, Moyamoya disease and syndrome, arterial dissection, and intracranial atherosclerosis.20,30⇓⇓⇓–34 This method can use high-resolution 2D or 3D imaging, frequently with pre- and postcontrast T1 or proton-attenuation sequences.20,32,35⇓⇓–38 In addition, high-resolution T2-weighted imaging can be used for multicontrast imaging.33,39⇓–41 In contradistinction to conventional angiographic imaging techniques that primarily evaluate the blood vessel lumen, such as conventional angiography, this approach provides information regarding the blood vessel wall itself, which is typically only 1–2 mm thick in proximal intracranial vessels.42, VWI has been described as black-blood imaging because it results in low signal in the vessel lumen, thereby aiding in the visualization of the blood vessel wall.


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