title: Challenging the diagnosis of primary angiitis of the central nervous system: a single-center retrospective study.
abstract: OBJECTIVE
  (1) To describe a series of adults assessed for suspected primary angiitis of the central nervous system (PACNS) and their final diagnosis; (2) to describe and compare presenting features of PACNS and reversible cerebral vasoconstriction syndrome (RCVS); and (3) to evaluate the specificity of the presenting features of RCVS.
  
  
  METHODS
  Patients evaluated at our institution between 2000 and 2008 for a possible CNS vasculitis and investigated by conventional angiography and/or brain biopsy were retrospectively analyzed. The inclusion criteria were a clinicoradiological presentation and cerebral angiography and/or brain biopsy raising the hypothesis of isolated cerebral vasculitis; and absence of identifiable etiology at the time of conventional angiogram and/or brain biopsy.
  
  
  RESULTS
  Among 58 cases evaluated, 37 met the inclusion criteria and 33 were included in the study. Thirteen patients had RCVS. Thunderclap headaches, the absence of a focal neurological deficit, a convexal subarachnoid hemorrhage and/or normal brain parenchyma on magnetic resonance imaging, and "string of beads" appearance on conventional angiography had high diagnostic value. Six patients had other noninflammatory vascular disorders (intracranial atherosclerosis, cryptogenic embolism, and genetic vasculopathy). Six patients had infection or malignancy. Eight patients were diagnosed with PACNS; their clinical presentation and disease course were heterogeneous. Brain biopsy was performed in 3 cases (positive in 1).
  
  
  CONCLUSIONS
  RCVS is an important differential diagnosis of CNS vasculitis. Its particular presentation should allow rapid identification in order to avoid pointless investigations and treatment. The frequent lack of histological proof and heterogeneous presentation of PACNS illustrated the nosological uncertainties of this label.
authors.affiliation: Service de Médecine Interne, Centre Hospitalier Universitaire de Nantes, Nantes, France. antoine.neel@univ-nantes.fr
authors.forename: Antoine
authors.initials: A
authors.lastname: Néel
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Auffray-Calvier
authors.forename: Benoit
authors.initials: B
authors.lastname: Guillon
authors.forename: Anne-Maelle
authors.initials: AM
authors.lastname: Fontenoy
authors.forename: Delphine
authors.initials: D
authors.lastname: Loussouarn
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.forename: Mohamed A
authors.initials: MA
authors.lastname: Hamidou
mesh_terms: D000328:Adult; D000368:Aged; D002533:Cerebral Angiography / Q000379:methods*; D015331:Cohort Studies; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed / Q000379:methods*; D020293:Vasculitis, Central Nervous System / Q000000981:diagnostic imaging* / Q000503:physiopathology; D020301:Vasospasm, Intracranial / Q000000981:diagnostic imaging* / Q000503:physiopathology; D055815:Young Adult