title: Reversible cerebral vasoconstriction syndromes and primary angiitis of the central nervous system: clinical, imaging, and angiographic comparison.
abstract: Reversible cerebral vasoconstriction syndromes (RCVS) and primary angiitis of the central nervous system (PACNS) are invariably considered in the differential diagnosis of new cerebral arteriopathies. However, prompt and accurate diagnosis remains challenging. Here we compared the features of 159 RCVS to 47 PACNS patients and developed criteria for prompt bedside diagnosis. Recurrent thunderclap headache (TCH), and single TCH combined with either normal neuroimaging, border zone infarcts, or vasogenic edema, have 100% positive predictive value for diagnosing RCVS or RCVS-spectrum disorders. In patients without TCH and positive angiography, neuroimaging can discriminate RCVS (no lesion) from PACNS (deep/brainstem infarcts). Ann Neurol 2016;79:882-894.
authors.affiliation: Massachusetts General Hospital and Harvard Medical School, Boston, MA.
authors.forename: Aneesh B
authors.initials: AB
authors.lastname: Singhal
authors.affiliation: Massachusetts General Hospital and Harvard Medical School, Boston, MA.|Neurology Department, Hacettepe University Hospitals, Ankara, Turkey.
authors.forename: Mehmet A
authors.initials: MA
authors.lastname: Topcuoglu
authors.affiliation: Department of Medicine, Yan Chai Hospital, Hong Kong, China.
authors.forename: Joshua W
authors.initials: JW
authors.lastname: Fok
authors.affiliation: Neurology Clinic, Ankara Numune Education and Research Hospital, Ankara, Turkey.
authors.forename: Oguzhan
authors.initials: O
authors.lastname: Kursun
authors.affiliation: Grady Memorial Hospital and Emory University School of Medicine, Atlanta, GA.
authors.forename: Raul G
authors.initials: RG
authors.lastname: Nogueira
authors.affiliation: Massachusetts General Hospital and Harvard Medical School, Boston, MA.
authors.forename: Matthew P
authors.initials: MP
authors.lastname: Frosch
authors.affiliation: Massachusetts General Hospital and Harvard Medical School, Boston, MA.
authors.forename: Verne S
authors.initials: VS
authors.lastname: Caviness
mesh_terms: D000328:Adult; D002533:Cerebral Angiography; D002561:Cerebrovascular Disorders / Q000175:diagnosis* / Q000000981:diagnostic imaging; D000072226:Computed Tomography Angiography; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D059906:Neuroimaging; D013577:Syndrome; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis* / Q000000981:diagnostic imaging; D014661:Vasoconstriction; D055815:Young Adult