S Calvez1, R Levy1,2,3,4, R Calvez5, C-J Roux1,2,3,4, D Grévent1,2,3,4, Y Purcell6, K Beccaria2,7, T Blauwblomme2,7, J Grill8, C Dufour8, F Bourdeaut2,9, F Doz2, M P Robert2,10, N Boddaert1,2,3,4, V Dangouloff-Ros11,2,3,4. 1. From the Pediatric Radiology Department (S.C., R.L., C.-J.R., D.G., N.B., V.D.-R.), Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France. 2. Paris University (R.L., C.-J.R., D.G., K.B., T.B., F.B., F.D., M.P.R., N.B., V.D.-R.), PRES Sorbonne Paris Cité, Paris, France. 3. Institut National de la Santé et de la Recherche Médicale U1000, (R.L., C.-J.R., D.G., N.B., V.D.-R.), Paris, France. 4. Institut Imagine (R.L., C.-J.R., D.G., N.B., V.D.-.R.), Unite Mixte de Recherche 1163, Paris, France. 5. Expert Biostatistician (R.C.), Gagny, France. 6. Radiology Department (Y.P.), Fondation Rothschild, Paris, France. 7. Pediatric Neurosurgery Department (K.B., T.B.), Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France. 8. Department of Pediatric and Adolescent Oncology (J.G., C.D.), Gustave Roussy Institute, Villejuif, France. 9. Oncology Center SIREDO (Care Innovation and Research for Children, Adolescents and Young Adults with Cancer) (F.B., F.D.), Institute Curie, ???????, France. 10. Ophthalmology Department (M.P.R.), Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France. 11. From the Pediatric Radiology Department (S.C., R.L., C.-J.R., D.G., N.B., V.D.-R.), Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France Volodia.dangouloff-ros@aphp.fr.
Abstract
BACKGROUND AND PURPOSE: Focal 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 METHODS: We 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. RESULTS: Thirty-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. CONCLUSIONS: Focal areas of high signal intensity are mostly small (<15 mm) abnormalities in the posterior fossa or capsular lenticular region. Our results suggest that the evolution of focal areas of high signal intensity is not related to puberty with a peak at the age of 7 years. Knowledge of the predictive evolution of focal areas of high signal intensity is essential in the follow-up of children with neurofibromatosis type 1.
BACKGROUND AND PURPOSE: Focal 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 METHODS: We 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. RESULTS: Thirty-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. CONCLUSIONS: Focal areas of high signal intensity are mostly small (<15 mm) abnormalities in the posterior fossa or capsular lenticular region. Our results suggest that the evolution of focal areas of high signal intensity is not related to puberty with a peak at the age of 7 years. Knowledge of the predictive evolution of focal areas of high signal intensity is essential in the follow-up of children with neurofibromatosis type 1.
Authors: K N North; V Riccardi; C Samango-Sprouse; R Ferner; B Moore; E Legius; N Ratner; M B Denckla Journal: Neurology Date: 1997-04 Impact factor: 9.910
Authors: Thibo Billiet; Burkhard Mädler; Felice D'Arco; Ronald Peeters; Sabine Deprez; Ellen Plasschaert; Alexander Leemans; Hui Zhang; Bea Van den Bergh; Mathieu Vandenbulcke; Eric Legius; Stefan Sunaert; Louise Emsell Journal: Neuroimage Clin Date: 2014-04-13 Impact factor: 4.881