title: Neurofibromatosis type 1: pathologic substrate of high-signal-intensity foci in the brain.
abstract: OBJECTIVE
  To investigate a correlation between pathologic and radiologic findings with regard to the characteristic high-signal-intensity foci seen on long repetition time (TR) magnetic resonance (MR) images of the brain in patients with neurofibromatosis type 1 (NF-1).
  
  
  METHODS
  Three girls with NF-1 and abnormal hyperintensities on long TR images of the brain underwent pathologic examination at autopsy.
  
  
  RESULTS
  Two 10-year-old girls had classic, focal hyperintensities in the internal capsules and globus pallidus regions, which have been associated with NF-1. The third patient, a neonate, had diffuse hyperintensity of the supratentorial and infratentorial white matter on T2-weighted MR images. Findings at histopathologic examination revealed spongiotic change in the tissue sections that correspond to the high-signal-intensity foci demonstrated on T2-weighted images.
  
  
  CONCLUSIONS
  Hyperintense foci seen on T2-weighted MR images appear to correspond to pathologic findings of areas of vacuolar or spongiotic change. The resultant fluid-filled vacuoles explain the occurrence of high signal intensity demonstrated on T2-weighted images.
authors.affiliation: Department of Radiology, Children's Hospital of Philadelphia, PA 19104, USA.
authors.forename: D P
authors.initials: DP
authors.lastname: DiPaolo
authors.forename: R A
authors.initials: RA
authors.lastname: Zimmerman
authors.forename: L B
authors.initials: LB
authors.lastname: Rorke
authors.forename: E H
authors.initials: EH
authors.lastname: Zackai
authors.forename: L T
authors.initials: LT
authors.lastname: Bilaniuk
authors.forename: A T
authors.initials: AT
authors.lastname: Yachnis
mesh_terms: D001921:Brain / Q000473:pathology*; D001932:Brain Neoplasms / Q000175:diagnosis / Q000473:pathology; D002648:Child; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000473:pathology