AJNR Am J Neuroradiol.

BACKGROUND AND PURPOSEFocal areas of high signal intensity are T2WI/T2-FLAIR hyperintensities frequently found on MR imaging of children diagnosed with neurofibromatosis type 1, often thought to regress spontaneously during adolescence or puberty. Due to the risk of tumor in this population, some focal areas of high signal intensity may pose diagnostic problems. The objective of this study was to assess the characteristics and temporal evolution of focal areas of high signal intensity in children with neurofibromatosis type 1 using long-term follow-up with MR imaging.MATERIALS AND METHODSWe retrospectively examined the MRIs of children diagnosed with neurofibromatosis type 1 using the National Institutes of Health Consensus Criteria (1987), with imaging follow-up of at least 4 years. We recorded the number, size, and surface area of focal areas of high signal intensity according to their anatomic distribution on T2WI/T2-FLAIR sequences. A generalized mixed model was used to analyze the evolution of focal areas of high signal intensity according to age, and separate analyses were performed for girls and boys.RESULTSThirty-nine patients (ie, 285 MR images) with a median follow-up of 7 years were analyzed. Focal areas of high signal intensity were found in 100% of patients, preferentially in the infratentorial white matter (35% cerebellum, 30% brain stem) and in the capsular lenticular region (22%). They measured 15 mm in 95% of cases. They appeared from the age of 1 year; increased in number, size, and surface area to a peak at the age of 7; and then spontaneously regressed by 17 years of age, similarly in girls and boys.CONCLUSIONSFocal areas of high signal intensity are mostly small (