Reversible cerebral vasoconstriction syndromes presenting with subarachnoid hemorrhage: a case series

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.