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

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: 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.

[1]  Derek M. Sorensen Reversible Cerebral Vasoconstriction Syndrome. , 2016, JAMA neurology.

[2]  Greg Zaharchuk,et al.  Arterial spin labeling MRI: Clinical applications in the brain , 2015, Journal of magnetic resonance imaging : JMRI.

[3]  R. Koide,et al.  A case of reversible cerebral vasoconstriction syndrome presenting with recurrent neurological deficits: Evaluation using noninvasive arterial spin labeling MRI , 2014, Clinical Neurology and Neurosurgery.

[4]  S. Jones,et al.  High-Resolution MRI Vessel Wall Imaging: Spatial and Temporal Patterns of Reversible Cerebral Vasoconstriction Syndrome and Central Nervous System Vasculitis , 2014, American Journal of Neuroradiology.

[5]  Seung-Chyul Hong,et al.  High-Resolution Magnetic Resonance Wall Imaging Findings of Moyamoya Disease , 2014, Stroke.

[6]  G. Schlaphoff,et al.  Reversible cerebral vasoconstriction syndrome following indomethacin , 2014, Cephalalgia : an international journal of headache.

[7]  Shih-Pin Chen,et al.  Hyperintense vessels: An early MRI marker of reversible cerebral vasoconstriction syndrome? , 2014, Cephalalgia : an international journal of headache.

[8]  H. Shimazaki,et al.  Unique combination of hyperintense vessel sign on initial FLAIR and delayed vasoconstriction on MRA in reversible cerebral vasoconstriction syndrome: A case report , 2014, Cephalalgia : an international journal of headache.

[9]  M. Sluzewski,et al.  How Often Is Thunderclap Headache Caused by the Reversible Cerebral Vasoconstriction Syndrome? , 2014, Headache.

[10]  H. Kabasawa,et al.  Evaluating middle cerebral artery atherosclerotic lesions in acute ischemic stroke using magnetic resonance T1-weighted 3-dimensional vessel wall imaging. , 2014, Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association.

[11]  P. Mathew,et al.  Reversible Cerebral Vasoconstriction Syndrome: Updates and New Perspectives , 2014, Current Pain and Headache Reports.

[12]  J. Biller,et al.  Reversible cerebral vasoconstriction syndrome with multivessel cervical artery dissections and a double aortic arch. , 2014, Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association.

[13]  Ye Qiao,et al.  Intracranial plaque enhancement in patients with cerebrovascular events on high-spatial-resolution MR images. , 2014, Radiology.

[14]  Bonnie H. Wang,et al.  Dramatic Intracerebral Hemorrhagic Presentations of Reversible Cerebral Vasoconstriction Syndrome: Three Cases and a Literature Review , 2014, Case reports in neurological medicine.

[15]  D. Kallmes,et al.  Clinical worsening in reversible cerebral vasoconstriction syndrome. , 2014, JAMA neurology.

[16]  D. Ramasamy,et al.  Reversible cerebral vasoconstriction syndrome (RCVS) in antiphospholipid antibody syndrome (APLA): the role of centrally acting vasodilators. Case series and review of literature , 2014, Clinical Rheumatology.

[17]  A. Singhal,et al.  Differentiating reversible cerebral vasoconstriction syndrome with subarachnoid hemorrhage from other causes of subarachnoid hemorrhage. , 2013, JAMA neurology.

[18]  J. Kim,et al.  High resolution MRI difference between moyamoya disease and intracranial atherosclerosis , 2013, European journal of neurology.

[19]  D. Segal,et al.  Call-Fleming syndrome: headache in a 16-year-old girl. , 2013, Pediatric neurology.

[20]  R. Hajj-Ali,et al.  Primary Angiitis of the Central Nervous System and Reversible Cerebral Vasoconstriction Syndrome , 2013, Current Atherosclerosis Reports.

[21]  A. Ducros L37. Reversible cerebral vasoconstriction syndrome: distinction from CNS vasculitis. , 2013, Presse medicale.

[22]  Kwo-whei Lee,et al.  Dual energy computed tomography angiography for the rapid diagnosis of reversible cerebral vasoconstriction syndromes: report of a case. , 2013, Acta neurologica Taiwanica.

[23]  G. Young,et al.  Cerebral Perfusion Imaging , 2012, Seminars in Neurology.

[24]  A. Andreou,et al.  Reversible Cerebral Vasoconstriction Syndrome: Treatment with Multiple Sessions of Intra-Arterial Nimodipine and Angioplasty , 2012, Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciences.

[25]  J. Gillard,et al.  MR Angiography and Imaging for the Evaluation of Middle Cerebral Artery Atherosclerotic Disease , 2012, American Journal of Neuroradiology.

[26]  J. Weinstein,et al.  Multimodal Imaging of Reversible Cerebral Vasoconstriction Syndrome: A Series of 6 Cases , 2012, American Journal of Neuroradiology.

[27]  D. Mikulis,et al.  Vessel Wall MRI to Differentiate Between Reversible Cerebral Vasoconstriction Syndrome and Central Nervous System Vasculitis: Preliminary Results , 2012, Stroke.

[28]  Zheng-yu Jin,et al.  Middle cerebral artery intraplaque hemorrhage: Prevalence and Clinical Relevance , 2012, Annals of neurology.

[29]  K. Digre,et al.  Repetitive use of intra-arterial verapamil in the treatment of reversible cerebral vasoconstriction syndrome , 2012, Journal of Clinical Neuroscience.

[30]  Shih-Pin Chen,et al.  Hyperintense vessels on flair imaging in reversible cerebral vasoconstriction syndrome , 2012, Cephalalgia : an international journal of headache.

[31]  S. Hetts,et al.  Reversible Cerebral Vasoconstriction Syndrome: Treatment with Combined Intra-Arterial Verapamil Infusion and Intracranial Angioplasty , 2011, American Journal of Neuroradiology.

[32]  Shih-Pin Chen,et al.  Reversible cerebral vasoconstriction syndrome: current and future perspectives , 2011, Expert review of neurotherapeutics.

[33]  Peter R Luijten,et al.  Intracranial Vessel Wall Imaging at 7.0-T MRI , 2011, Stroke.

[34]  A. Singhal,et al.  Reversible cerebral vasoconstriction syndromes: analysis of 139 cases. , 2011, Archives of neurology.

[35]  Michael Schär,et al.  Intracranial arterial wall imaging using three‐dimensional high isotropic resolution black blood MRI at 3.0 Tesla , 2011, Journal of magnetic resonance imaging : JMRI.

[36]  A. Singhal,et al.  Primary angiitis of the CNS , 2011, The Lancet Neurology.

[37]  S. Ansari,et al.  Reversible cerebral vasoconstriction syndromes presenting with subarachnoid hemorrhage: a case series , 2011, Journal of NeuroInterventional Surgery.

[38]  A. Straube,et al.  Intra-arterial application of nimodipine in reversible cerebral vasoconstriction syndrome: A diagnostic tool in select cases? , 2011, Cephalalgia : an international journal of headache.

[39]  Zheng-yu Jin,et al.  In vivo high-resolution MR imaging of symptomatic and asymptomatic middle cerebral artery atherosclerotic stenosis. , 2010, Atherosclerosis.

[40]  B. Shia,et al.  Magnetic resonance angiography in reversible cerebral vasoconstriction syndromes , 2009, Annals of neurology.

[41]  A. Rabinstein Atraumatic convexal subarachnoid hemorrhage: Clinical presentation, imaging patterns, and etiologies , 2010 .

[42]  A. Straube,et al.  Reversible Cerebral Vasoconstriction Syndrome: A Complicated Clinical Course Treated with Intra-Arterial Application of Nimodipine , 2009, Cephalalgia : an international journal of headache.

[43]  D. Mikulis,et al.  Intracranial arterial wall imaging using high-resolution 3-tesla contrast-enhanced MRI , 2009, Neurology.

[44]  T. Tominaga,et al.  Middle cerebral artery plaque imaging using 3-Tesla high-resolution MRI , 2008, Journal of Clinical Neuroscience.

[45]  J. De Keyser,et al.  An often unrecognized cause of thunderclap headache: reversible cerebral vasoconstriction syndrome , 2008, The Journal of Headache and Pain.

[46]  B. Shia,et al.  Transcranial color doppler study for reversible cerebral vasoconstriction syndromes , 2008, Annals of neurology.

[47]  Peter M. Rothwell,et al.  Vessel Wall Contrast Enhancement: A Diagnostic Sign of Cerebral Vasculitis , 2008, Cerebrovascular Diseases.

[48]  R. Porcher,et al.  The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. , 2007, Brain : a journal of neurology.

[49]  J. De Keyser,et al.  Pitfalls in the diagnosis of reversible cerebral vasoconstriction syndrome and primary angiitis of the central nervous system , 2007, European journal of neurology.

[50]  S. Kasner,et al.  Reversible Cerebral Vasoconstriction Syndrome Associated with Subarachnoid Hemorrhage , 2007, Neurocritical care.

[51]  A. Singhal,et al.  CT angiography and diffusion-perfusion MR imaging in a patient with ipsilateral reversible cerebral vasoconstriction after carotid endarterectomy. , 2007, AJNR. American journal of neuroradiology.

[52]  C. Nickele,et al.  Severe reversible cerebral vasoconstriction syndrome mimicking aneurysmal rupture and vasospasm , 2007, Neurocritical care.

[53]  A. Singhal,et al.  Narrative Review: Reversible Cerebral Vasoconstriction Syndromes , 2007, Annals of Internal Medicine.

[54]  A. Singhal,et al.  Postpartum angiopathy and other cerebral vasoconstriction syndromes , 2005, Neurocritical care.

[55]  C. Oppenheim,et al.  Evaluation of Hyperintense Vessels on FLAIR MRI for the Diagnosis of Multiple Intracerebral Arterial Stenoses , 2003, Stroke.

[56]  A. Furlan,et al.  Benign angiopathy of the central nervous system: cohort of 16 patients with clinical course and long-term followup. , 2002, Arthritis and rheumatism.

[57]  S. Kamath Observations on the length and diameter of vessels forming the circle of Willis. , 1981, Journal of anatomy.