Reversible cerebral vasoconstriction syndromes presenting with subarachnoid hemorrhage: a case series
- 1Departments of Radiology, Neurology and Neurosurgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
- 2Department of Radiology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA
- 3Departments of Radiology and Neurosurgery, Johns Hopkins Hospital, Baltimore, Maryland, USA
- Correspondence to Sameer A Ansari, M.D., Ph.D., Neurointerventional Surgery, Departments of Radiology, Neurology, and Neurosurgery, Northwestern University Feinberg School of Medicine, 676 N. St Clair Street, Suite 800, Chicago, IL 60611-2927, USA;
- Received 2 November 2010
- Revised 18 December 2010
- Accepted 22 December 2010
- Published Online First 4 February 2011
Background and purpose Reversible cerebral vasoconstriction syndromes (RCVS) represent a heterogeneous group of cerebrovascular disease characterized by acute presentations and transient segmental narrowing of the distal intracranial arteries. A series of patients with RCVS were studied to better understand the clinical and imaging characteristics of this rare pathology.
Methods A retrospective study was performed on patients that met inclusion criteria for a diagnosis of RCVS. Pertinent clinical and laboratory data, initial and follow-up imaging, treatment and outcomes were studied.
Results 11 patients (10 women, mean age 42 years) diagnosed with RCVS presented with acute onset of severe headache, neurological symptoms and subarachnoid hemorrhage (SAH). Cross sectional imaging (CT/MRI) identified presentations of cortical SAH (n=9) and/or acute infarcts (n=3). Initial cerebral angiography (digital subtraction angiography n=10 or MR angiography n=1) confirmed diffuse vasoconstriction involving the intracranial vasculature. Rheumatological panel (n=9) and CSF analysis (n=8) were not supportive of vasculitis in any patient. In nearly all cases, reversal of vasoconstriction was noted on follow-up cerebral angiography with early resolution in less than 3 months.
Conclusions RCVS classically presents with ‘thunderclap’ headaches and neurological symptoms but cortical SAH is not an uncommon presentation with a unique and focal distribution overlying the cerebral sulci. Although the initial clinical and angiographic appearance of RCVS may be confused for vasospasm related to aneurysmal SAH or primary angiitis of the CNS, its clinical, laboratory and imaging features assist in diagnosis.
SA and TJR contributed equally to this work.
Competing interests None.
Ethics approval This study was conducted with the approval of the Institutional Review Board, University of Michigan Health System.
Provenance and peer review Not commissioned; externally peer reviewed.