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

Fellows’ Journal Club

Editor’s Comment

Noninvasive vascular imaging, such as transcranial Doppler sonography and MR angiography, has played an increasingly important role is diagnosing this condition, though conventional angiography remains the reference standard for the evaluation of cerebral artery vasoconstriction.

Summary

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).
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).

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.

 

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