Back
Full-text
Journal article

Focal Cerebral Arteriopathy of Childhood: Novel Severity Score and Natural History.

  • Fullerton HJ From the Department of Neurology (H.J.F., N.K.H.), University of California, San Francisco.
  • Stence N Department of Radiology (N.S.), University of Colorado, Denver.
  • Hills NK From the Department of Neurology (H.J.F., N.K.H.), University of California, San Francisco.
  • Jiang B Division of Neuroradiology, Department of Radiology, Stanford University, CA (B.J., M.W.).
  • Amlie-Lefond C Department of Neurology, University of Washington, Seattle (C.A.-L.).
  • Bernard TJ Department of Pediatrics (T.J.B.), University of Colorado, Denver.
  • Friedman NR Center for Pediatric Neurosciences, Neurological Institute, Cleveland Clinic, OH (N.R.F.).
  • Ichord R Department of Neurology, Children's Hospital of Philadelphia, Perelman School of Medicine of the University of Pennsylvania (R.I.).
  • Mackay MT Department of Paediatrics, Royal Children's Hospital, Murdoch Children's Research Institute, University of Melbourne, Victoria, Australia (M.T.M.).
  • Rafay MF Department of Pediatrics and Child Health, Children's Hospital, University of Manitoba, Winnipeg, Canada (M.F.R.).
  • Chabrier S INSERM, UMR1059 Sainbiose, University of Lyon, CHU Saint-Étienne, France (S.C.).
  • Steinlin M Department of Pediatric Neurology, University Children's Hospital, University of Bern, Switzerland (M.S.).
  • Elkind MSV Department of Epidemiology, Mailman School of Public Health (M.S.V.E.), Columbia University, New York, NY.
  • deVeber GA Division of Neurology, Hospital for Sick Children, Toronto, Canada (G.A.D.).
  • Wintermark M Division of Neuroradiology, Department of Radiology, Stanford University, CA (B.J., M.W.).
Show more…
  • 2018-10-26
Published in:
  • Stroke. - 2018
brain ischemia
cerebrovascular disorders
child
follow-up studies
humans
Adolescent
Anterior Cerebral Artery
Brain Infarction
Carotid Artery, Internal
Carotid Stenosis
Cerebral Angiography
Cerebral Arterial Diseases
Child
Child, Preschool
Computed Tomography Angiography
Disease Progression
Female
Humans
Infant
Magnetic Resonance Angiography
Male
Middle Cerebral Artery
Posterior Cerebral Artery
Severity of Illness Index
Stroke
English Background and Purpose- Focal cerebral arteriopathy (FCA)-a common cause of arterial ischemic stroke in previously healthy children-often progresses over days to weeks, increasing the risk of recurrent stroke. We developed a novel severity scoring system designed to quantify FCA progression and correlate with clinical outcomes. Methods- The VIPS study (Vascular Effects of Infection in Pediatric Stroke) prospectively enrolled 355 children with arterial ischemic stroke (2010-2014), including 41 with centrally confirmed FCA. Two neuroradiologists independently reviewed FCA cerebrovascular imaging, assigning a graded severity score of zero (no involvement) to 4 (occlusion) to individual arterial segments. The FCA severity score (FCASS) was the unweighted sum. In an iterative process, we modeled scores derived from different combinations of arterial segments to identify the model that optimized correlation with clinical outcome, simplicity, and reliability. Results- The optimal FCASS summed scores from 5 arterial segments: supraclinoid internal carotid artery, A1, A2, M1, and M2. The median (interquartile range) baseline FCASS was 4 (2-6). Of 33 children with follow-up imaging, the maximum FCASS (at any time point) was 7 (5-9). Twenty-four (73%) had FCA progression on follow-up with their maximum FCASS at a median of 8 (5-35.5) days poststroke; their median FCASS increase was 4 (2.5-6). FCASS did not correlate with recurrent arterial ischemic stroke. Maximum (but not baseline) FCASS correlated with 1-year pediatric stroke outcome measures ( P=0.037). Conclusions- Our novel scoring system for FCA severity correlates with neurological outcomes in the VIPS cohort and provides a tool for FCA treatment trials under development.
Language
  • English
Open access status
green
Identifiers
Persistent URL
https://sonar.ch/global/documents/170079
Statistics
Document views: 22 File downloads:
  • fulltext.pdf: 0