Multimodal Imaging of Reversible Cerebral Vasoconstriction Syndrome: A Series of 6 Cases

Abstract

SUMMARY: RCVS is a clinical condition of recurrent severe headaches that may be associated with ischemic or hemorrhagic stroke and that is defined by the presence of segmental vasoconstriction in multiple cerebral arteries. The angiographic appearance resembles vasculitis, except that the abnormalities resolve during the course of several months. Because the treatment of RCVS differs from that for vasculitis, radiologists must understand the clinical and radiologic features so as to better guide imaging algorithms and facilitate diagnosis. We present a series of 6 cases of RCVS that highlight the imaging features across multiple modalities.ABBREVIATIONS: IPH ϭ intraparenchymal hemorrhage; MIP ϭ maximum intensity projection; NSAID ϭ nonsteroidal anti-inflammatory drug; PACNS ϭ primary angiitis of the CNS; PRES ϭ posterior reversible encephalopathy syndrome; RCVS ϭ reversible cerebral vasoconstriction syndrome; SDH ϭ subdural hematoma; TCD ϭ transcranial Doppler R CVS is an under-recognized clinical-radiologic entity characterized by a history of sudden severe headaches, sometimes associated with ischemic or hemorrhagic stroke, focal neurologic deficits, or seizures. First described by Call et al in 19881 , RCVS encompasses a wide variety of entities previously described by other names, including postpartum angiopathy, migrainous vasospasm, migrainous stroke, druginduced angiopathy, and benign angiopathy of the CNS. 2,3The condition is defined by reversible segmental cerebral vasoconstriction on angiography.1-3 The angiographic findings are similar to those of other vasculopathies, including PACNS.4 Unlike PACNS, the vascular abnormalities of RCVS resolve within several months. 1,2,5,6 RCVS typically occurs following exposure to a trigger, commonly sympathomimetic or vasoactive agents, including amphetamines, phenylpropanolamine, pseudoephedrine, serotonergic antidepressants, nicotine, caffeine, cannabis, and triptan-or ergot-containing medications.2,7-9 Other triggers include the peripartum period, 10 eclampsia, strenuous physical activity, bathing or showering, sexual activity, and binge alcohol drinking.2 Most patients with RCVS are young and middle-aged women. 3Patients with RCVS exhibit a range of parenchymal abnormalities, including convexal SAH, IPH, ischemic infarcts, and PRES.2,9,11-13 Alternatively, imaging findings may be normal.2,9 Findings of CSF analysis are usually normal or nearnormal.3 Because of the overlap with other causes of headache and stroke in adults and because the vascular abnormalities early in the course of the disease may be subtle or absent, the diagnosis of RCVS is easily missed. Early recognition of RCVS allows appropriate clinical management aimed at reducing the frequency, duration, and severity of vascular complications, primarily through identification and removal of triggers. Because management and outcome of RCVS differ from those of other vasculopathies, it is critical that radiologists recognize its typical imaging appearance, time course, and clinical features. Case Series...

Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical Course

“…Initially, patients presenting with cerebral vasoconstriction were thought to have unique disease entities, depending on the given clinical scenario and specialist treating the patient (Table 1). [4][5][6][8][9][10][11] The common features of these cases, including clinical presentation with severe headache, reversibility of angiographic findings, and lack of histologic abnormalities on arterial biopsy, were not well appreciated or understood.…”

“…[1][2][3][4][5] RCVS is not a single disease entity but should be considered a common presentation of multiple disorders characterized by reversible vasoconstriction of the cerebral vasculature. 3,[6][7][8] The term "RCVS" now encompasses what was previously thought to be a group of distinct clinical entities, including Call-Fleming syndrome, thunderclap headache, and postpartum angiopathy.…”

“…3,[6][7][8] The term "RCVS" now encompasses what was previously thought to be a group of distinct clinical entities, including Call-Fleming syndrome, thunderclap headache, and postpartum angiopathy. [4][5][6][8][9][10][11] Timely and accurate diagnosis of RCVS is essential to ensuring appropriate patient care and avoiding unnecessary diagnostic tests. However, the diagnosis can be challenging because its signs and symptoms can overlap those of better known disorders of the central nervous system, including aneurysmal subarachnoid hemorrhage and primary angiitis of the CNS.…”

Radiological Diagnosis and Differential Diagnosis of Headache

“…Follow-up should entail MR imaging ( • " Table 3), since in such cases diffusion-weighted images can reliably detect infarcts as a possible complication [22]. Due to method-related higher sensitivity required to detect diameter variations, imaging of the intracranial arteries should employ TOF angiography compared to contrast-enhanced angiography (CE MRA) [23].…”

Fulminant reversible cerebral vasoconstriction syndrome in post-partum female

“…Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical and radiologic syndrome whose primary features include the hyper-acute onset of severe headache and segmental vasoconstriction of cerebral arteries that resolves by 3 months. [1][2][3][4][5] Common imaging findings are diffuse segmental cerebral vasoconstriction of the intracranial internal carotid arteries, basilar artery and arteries of the circle of Willis that spontaneously resolves in weeks to months. Although the patho-physiology of RCVS remains unknown, the prevailing hypothesis involves a transient disturbance in vascular tone.…”