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PEDIATRICS

Angiography of Primary Central Nervous System Angiitis of Childhood: Conventional Angiography versus Magnetic Resonance Angiography at Presentation

R.I. Aviva,f,h, S.M. Benselerb,d, G. DeVeberc,d,f,h, E.D. Silvermanb,d,f,g,h, P.N. Tyrrellb, L.M. Tsangb and D. Armstronga,e,h

a Division of Neuroradiology, Hospital for Sick Children, Toronto, Ontario, Canada
b Division of Rheumatology, Hospital for Sick Children, Toronto, Ontario, Canada
c Division of Neurology, Hospital for Sick Children, Toronto, Ontario, Canada
d Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada
e Department of Radiology, Hospital for Sick Children, Toronto, Ontario, Canada
f Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada
g Department of Immunology, University of Toronto, Toronto, Ontario, Canada
h Department of Radiology, University of Toronto, Toronto, Ontario, Canada

Address correspondence to Dr. Richard Aviv, Sunnybrook Health Sciences Center, 2075 Bayview Ave, Toronto, ON M4N 3M5, Canada; e-mail: richardaviv{at}lineone.net

BACKGROUND AND PURPOSE: To systematically analyze conventional angiographic (CA) features of children with primary central nervous system angiitis (cPACNS), to compare and correlate CA and MR angiography (MRA) lesion characteristics, and to define the sensitivity and specificity of MRA with CA as a reference standard.

METHODS: A retrospective, single-center cohort study of consecutive patients with cPACNS was performed. Patients with CA and MRA studies at diagnosis were included. Imaging studies were blindly reviewed by 2 neuroradiologists using a standard analysis protocol. CA and MRA studies were compared using nonparametric analysis.

RESULTS: Of 45 patients with MRA at diagnosis, there were 25 for whom CA and MRA studies were performed within 1 month of each other. These comprised the study group. The CA distribution of lesions was multifocal (76%) and proximal (86%) (P < .05) with a trend toward unilaterality (P = .06) with anterior circulation involvement (P = .08). The sensitivity and specificity of MRA for CA abnormality was 70% and 98%, respectively. There was no significant difference between MRA and CA for lesion detection or characterization (P = .87), and the modalities showed a fair correlation ({kappa} = 0.4).

CONCLUSION: Angiographic lesions are multifocal and occur proximally and unilaterally within the anterior circulation. There is no significant difference in the ability of MRA to detect and characterize lesions when compared with CA.




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