title: A novel approach to diagnose reversible cerebral vasoconstriction syndrome: a case series.
abstract: BACKGROUND
  Reversible cerebral vasoconstriction syndrome (RCVS) is classically diagnosed based on the presence of severe thunderclap headache, focal neurologic symptoms, and the radiographic findings of reversible diffuse segmental cerebral vasoconstriction. We present a diagnostic test that may assist in the clinical diagnosis and facilitate treatment.
  
  
  METHODS
  From October 1, 2010, to August 1, 2013, we identified consecutive patients who presented with a presumptive diagnosis of RCVS and underwent cerebral diagnostic angiography with intra-arterial (IA) vasodilator therapy. Medical records including clinical presentation, radiographic, and angiographic images were all reviewed.
  
  
  RESULTS
  We identified a total of 7 patients (4 females; age range, 22-56; mean, 45 years) who met our inclusion criteria. Four patients received a combination of milrinone and nicardipine infusion either in the internal carotid arteries or in the left vertebral artery; the remaining patients received IA therapy solely with either nicardipine or milrinone. Five patients had a positive angiographic response, defined as significant improvement or resolution of the blood vessels irregularities. All 5 patients had a definite discharge diagnosis of RCVS. The remaining 2 patients had a negative angiographic response and based on their clinical and radiographic course had a final diagnosis of intracranial atherosclerotic disease.
  
  
  CONCLUSIONS
  Our small case series suggest that IA administration of vasodilators is safe and may aid in distinguishing vasodilator responsive syndromes such as RCVS from other causes. Further study is required with long-term clinical outcome to determine the utility of this diagnostic test.
authors.affiliation: Department of Neurology, Emory University School of Medicine, Atlanta, GA.
authors.forename: Tareq
authors.initials: T
authors.lastname: Kass-Hout
authors.affiliation: Department of Neurology, Emory University School of Medicine, Atlanta, GA.
authors.forename: Omar
authors.initials: O
authors.lastname: Kass-Hout
authors.affiliation: Department of Neurology, Emory University School of Medicine, Atlanta, GA.
authors.forename: Chung-Huan J
authors.initials: CH
authors.lastname: Sun
authors.affiliation: Humanitarian Tracker, Washington, DC.
authors.forename: Taha
authors.initials: T
authors.lastname: Kass-Hout
authors.affiliation: Department of Neurology, Emory University School of Medicine, Atlanta, GA; Department of Neurosurgery, Emory University School of Medicine, Atlanta, GA.
authors.forename: Pankajavalli
authors.initials: P
authors.lastname: Ramakrishnan
authors.affiliation: Department of Neurology, Emory University School of Medicine, Atlanta, GA.
authors.forename: Fadi
authors.initials: F
authors.lastname: Nahab
authors.affiliation: Department of Neurology, Emory University School of Medicine, Atlanta, GA.
authors.forename: Raul
authors.initials: R
authors.lastname: Nogueira
authors.affiliation: Department of Neurosurgery, Wellstar Neurosurgery, Kennestone Hospital, Marietta, GA. Electronic address: Guptar31@gmail.com.
authors.forename: Rishi
authors.initials: R
authors.lastname: Gupta
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D051270:Headache Disorders, Primary / Q000175:diagnosis*; D006801:Humans; D007261:Infusions, Intra-Arterial; D008297:Male; D008875:Middle Aged; D020105:Milrinone / Q000008:administration & dosage; D009529:Nicardipine / Q000008:administration & dosage; D014661:Vasoconstriction / Q000187:drug effects*; D014665:Vasodilator Agents / Q000008:administration & dosage; D020301:Vasospasm, Intracranial / Q000175:diagnosis*; D055815:Young Adult
chemical_list: D014665:Vasodilator Agents; D009529:Nicardipine; D020105:Milrinone
keywords: Reversible cerebral vasoconstriction syndrome; central nervous system vasculitis; intra-arterial vasodilators; intracranial atherosclerotic disease; moyamoya disease