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Research ArticlePediatrics

Focal Areas of High Signal Intensity in Children with Neurofibromatosis Type 1: Expected Evolution on MRI

S. Calvez, R. Levy, R. Calvez, C.-J. Roux, D. Grévent, Y. Purcell, K. Beccaria, T. Blauwblomme, J. Grill, C. Dufour, F. Bourdeaut, F. Doz, M.P. Robert, N. Boddaert and V. Dangouloff-Ros
American Journal of Neuroradiology September 2020, 41 (9) 1733-1739; DOI: https://doi.org/10.3174/ajnr.A6740
S. Calvez
aFrom 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
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R. Levy
aFrom 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
bParis 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
cInstitut National de la Santé et de la Recherche Médicale U1000, (R.L., C.-J.R., D.G., N.B., V.D.-R.), Paris, France
dInstitut Imagine (R.L., C.-J.R., D.G., N.B., V.D.-.R.), Unite Mixte de Recherche 1163, Paris, France
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R. Calvez
eExpert Biostatistician (R.C.), Gagny, France
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C.-J. Roux
aFrom 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
bParis 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
cInstitut National de la Santé et de la Recherche Médicale U1000, (R.L., C.-J.R., D.G., N.B., V.D.-R.), Paris, France
dInstitut Imagine (R.L., C.-J.R., D.G., N.B., V.D.-.R.), Unite Mixte de Recherche 1163, Paris, France
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D. Grévent
aFrom 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
bParis 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
cInstitut National de la Santé et de la Recherche Médicale U1000, (R.L., C.-J.R., D.G., N.B., V.D.-R.), Paris, France
dInstitut Imagine (R.L., C.-J.R., D.G., N.B., V.D.-.R.), Unite Mixte de Recherche 1163, Paris, France
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Y. Purcell
fRadiology Department (Y.P.), Fondation Rothschild, Paris, France
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K. Beccaria
bParis 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
gPediatric Neurosurgery Department (K.B., T.B.), Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France
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T. Blauwblomme
bParis 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
gPediatric Neurosurgery Department (K.B., T.B.), Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France
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J. Grill
hDepartment of Pediatric and Adolescent Oncology (J.G., C.D.), Gustave Roussy Institute, Villejuif, France
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C. Dufour
hDepartment of Pediatric and Adolescent Oncology (J.G., C.D.), Gustave Roussy Institute, Villejuif, France
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F. Bourdeaut
bParis 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
iOncology Center SIREDO (Care Innovation and Research for Children, Adolescents and Young Adults with Cancer) (F.B., F.D.), Institute Curie, ???????, France.
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F. Doz
bParis 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
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M.P. Robert
bParis 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
jOphthalmology Department (M.P.R.), Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France
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N. Boddaert
aFrom 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
bParis 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
cInstitut National de la Santé et de la Recherche Médicale U1000, (R.L., C.-J.R., D.G., N.B., V.D.-R.), Paris, France
dInstitut Imagine (R.L., C.-J.R., D.G., N.B., V.D.-.R.), Unite Mixte de Recherche 1163, Paris, France
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V. Dangouloff-Ros
aFrom 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
bParis 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
cInstitut National de la Santé et de la Recherche Médicale U1000, (R.L., C.-J.R., D.G., N.B., V.D.-R.), Paris, France
dInstitut Imagine (R.L., C.-J.R., D.G., N.B., V.D.-.R.), Unite Mixte de Recherche 1163, Paris, France
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    FIG 1.

    The distribution of FASI on all MR imaging examinations in size classes by clusters. Seventy-nine percent of FASI were <10 mm. FASI of > 20 mm appeared only for females (n = 7 females, 2 of whom were outliers) and only in the capsular lenticular region, brain stem, and cerebellum clusters.

  • FIG 2.
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    FIG 2.

    Temporal evolution of scatterplots of the total FASI number of all MRIs (A) and total FASI surface area of all MRIs (B). Uppercase letters are for females, and lowercase letters are for males. The regression curve is 3°. The x-axis represents age, the y-axis represents the number of FASI (A), and the surface area is in square millimeters (B). Female and male regression curves are descriptive.

  • FIG 3.
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    FIG 3.

    Temporal evolution of FASI on T2-FLAIR-weighted images. Axial T2-FLAIR sequences show capsular lenticular (A) and infratentorial white matter (C) FASI in a 6-year-old patient, then follow-up MR imaging in a 10-year-old patient with almost complete disappearance of these FASI (B and D).

  • FIG 4.
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    FIG 4.

    Age of the patients at the time of the peak of FASI surface. The mean age at the peak was 7.2 ± 2.8 years. The 3 patients with a peak age after 13 years had their first MR imaging after the age of 7 years (7.5, 13, and 13 years).

  • FIG 5.
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    FIG 5.

    Hippocampal hyperintensity on T2 FLAIR image. Coronal T2 FLAIR sequence shows the diffuse homogeneous and symmetric hyperintensity of both hippocampi. FASI are also seen in the globus pallidus on both sides.

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S. Calvez, R. Levy, R. Calvez, C.-J. Roux, D. Grévent, Y. Purcell, K. Beccaria, T. Blauwblomme, J. Grill, C. Dufour, F. Bourdeaut, F. Doz, M.P. Robert, N. Boddaert, V. Dangouloff-Ros
Focal Areas of High Signal Intensity in Children with Neurofibromatosis Type 1: Expected Evolution on MRI
American Journal of Neuroradiology Sep 2020, 41 (9) 1733-1739; DOI: 10.3174/ajnr.A6740

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Focal Areas of High Signal Intensity in Children with Neurofibromatosis Type 1: Expected Evolution on MRI
S. Calvez, R. Levy, R. Calvez, C.-J. Roux, D. Grévent, Y. Purcell, K. Beccaria, T. Blauwblomme, J. Grill, C. Dufour, F. Bourdeaut, F. Doz, M.P. Robert, N. Boddaert, V. Dangouloff-Ros
American Journal of Neuroradiology Sep 2020, 41 (9) 1733-1739; DOI: 10.3174/ajnr.A6740
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