Reduced cerebral arterial spin-labeled perfusion in children with neurofibromatosis type 1

AJNR Am J Neuroradiol. 2013 Sep;34(9):1823-8. doi: 10.3174/ajnr.A3649. Epub 2013 Jun 13.

Abstract

Background and purpose: Neurofibromatosis type 1 is associated with increased risk for stroke, cerebral vasculopathy, and neurocognitive deficits, but underlying hemodynamic changes in asymptomatic children remain poorly understood. We hypothesized that children with neurofibromatosis type 1 have decreased cerebral blood flow.

Materials and methods: Arterial spin-labeled CBF was measured in 14 children with neurofibromatosis type 1 (median age, 9.7 years; mean, 10.2 years; range, 22 months to 18 years) and compared with age-matched control subjects on 3T MR imaging. Three-dimensional pseudocontinuous spin-echo arterial spin-labeled technique was used. Measurements were obtained at cortical gray matter of bilateral cerebral hemispheres and centrum semiovale by use of the ROI method. Comparison by Mann-Whitney test was used, with Bonferroni-adjusted P values ≤.004 judged as significant.

Results: We identified 7 of 12 areas with significantly diminished arterial spin-labeled CBF in patients with neurofibromatosis type 1 compared with control subjects. These areas included the anterior cingulate gyrus (P = .001), medial frontal cortex (P = .004), centrum semiovale (P = .004), temporo-occipital cortex (P = .002), thalamus (P = .001), posterior cingulate gyrus (P = .002), and occipital cortex (P = .001). Among patients with neurofibromatosis type 1, there were no significant differences in these regions on the basis of the presence of neurofibromatosis type 1 spots or neurocognitive deficits.

Conclusions: Reduced cerebral perfusion was seen in children with neurofibromatosis type 1, particularly in the posterior circulation and the vascular borderzones of the middle and posterior cerebral arteries.

MeSH terms

  • Blood Flow Velocity
  • Cerebral Arteries / pathology*
  • Cerebral Arteries / physiopathology*
  • Cerebrovascular Circulation*
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Magnetic Resonance Angiography
  • Male
  • Neurofibromatosis 1 / pathology*
  • Neurofibromatosis 1 / physiopathology*
  • Reproducibility of Results
  • Sensitivity and Specificity
  • Spin Labels

Substances

  • Spin Labels