Reversible Cerebral Vasoconstriction Syndrome, Part 2: Diagnostic Work-Up, Imaging Evaluation, and Differential Diagnosis

Abstract

The diagnostic evaluation of a patient with reversible cerebral vasoconstriction syndrome integrates clinical, laboratory, and radiologic findings. Imaging plays an important role by confirming the presence of cerebral vasoconstriction; monitoring potential complications such as ischemic stroke; and suggesting alternative diagnoses, including CNS vasculitis and aneurysmal subarachnoid hemorrhage. Noninvasive vascular imaging, including transcranial Doppler sonography and MR angiography, has played an increasingly important role in this regard, though conventional angiography remains the criterion standard for the evaluation of cerebral artery vasoconstriction. 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.

Fig 1.

A 55-year-old woman who presented with severe headache and developed left-sided weakness. DWI (A) shows multifocal infarcts involving the centrum semiovale and left posterior parietal lobe. On coronal 3D reformatted TOF MRA (B), there is diffuse narrowing of the bilateral middle and anterior cerebral arteries (white arrowheads). Parasagittal postcontrast T1 high-resolution VWI of the M1 arterial segment of the left MCA (C) shows mild wall thickening and minimal enhancement (similar findings were noted in the right M1 arterial segment, not shown). The patient was diagnosed with RCVS, with subsequent resolution of cerebral vasoconstriction (D).

Fig 2.

A 35-year-old man with a history of Behçet vasculitis who presented with left-sided weakness. DWI (A) demonstrates an acute infarct involving the right thalamus and posterior limb of the internal capsule (white arrow). Coronal 3D reformat of TOF MRA (B) reveals irregularity and narrowing of the M1 arterial segment of the right MCA (white arrow) and occlusion or high-grade stenosis of the P1 arterial segment of the right PCA (curved white arrow). On axial T1 postcontrast high-resolution VWI (C), there is prominent enhancement and enlargement of the right posterior cerebral artery (white arrow). Sagittal T1 postcontrast VWI (D) demonstrates typical tram-track, circumferential enhancement of the right M1 MCA (white arrow), consistent with vasculitic inflammation.

Fig 3.

A 55-year-old woman (the same patient as in Fig 1) with RCVS complicated by ischemic infarcts. 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). These areas of perfusion abnormality correspond, in part, to regions of restriction diffusion/ischemic infarct on DWI (D).

Fig 4.

A 59-year-old man with a history of seizures, who was subsequently found to have multifocal infarcts in several vascular territories (not shown). 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. On axial T1 precontrast high-resolution VWI (C), there is intrinsic T1 mural hyperintensity in involved MCA (white arrows) and anterior cerebral artery branches. On axial T1 postcontrast high-resolution VWI (D), there are accompanying areas of eccentric vessel wall enhancement (white arrow, D).