Incidental parenchymal magnetic resonance imaging findings in the brains of patients with neurofibromatosis type 2

Purpose: Whereas T2 hyperintensities known as NF-associated bright spots are well described in patients with neurofibromatosis type I (NF-1), there is a paucity of data on incidental findings in patients with neurofibromatosis type II (NF-2). We aim to characterize unexplained imaging findings in th...

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Main Authors: Wendy S. Vargas, Linda A. Heier, Fausto Rodriguez, Amanda Bergner, Kaleb Yohay
Format: Article
Language:English
Published: Elsevier 2014-01-01
Series:NeuroImage: Clinical
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2213158213001691
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author Wendy S. Vargas
Linda A. Heier
Fausto Rodriguez
Amanda Bergner
Kaleb Yohay
author_facet Wendy S. Vargas
Linda A. Heier
Fausto Rodriguez
Amanda Bergner
Kaleb Yohay
author_sort Wendy S. Vargas
collection DOAJ
description Purpose: Whereas T2 hyperintensities known as NF-associated bright spots are well described in patients with neurofibromatosis type I (NF-1), there is a paucity of data on incidental findings in patients with neurofibromatosis type II (NF-2). We aim to characterize unexplained imaging findings in the brains of patients with NF-2. Materials and methods: This study is retrospective, HIPAA-compliant and approved by the institutional review board. 34 patients with NF-2 underwent brain magnetic resonance imaging (MRI) between January 2000 and December 2012. T2 and T1-weighted imaging characteristics, diffusion weighted imaging (DWI) characteristics, and enhancement patterns were analyzed by visual inspection. Clinical information at time of imaging was available for all patients. Neuropathologic data was available for one patient. Results: We found unexplained T2 hyperintensities present on initial imaging in 23/34 patients (67%). Of the 23 patients with unexplained MRI findings, 15 (65%) had wedge-shaped T2 hyperintensities in the subcortical white matter extending to the cortex suggestive of a cortical dysplasia. 3 additional cases (17%) had a lesion within the cerebellum suggestive of a neuronal migration anomaly. In one patient where the MRI was suggestive of focal cortical dysplasia, histopathologic analysis revealed dysplastic glial foci without other alterations of cortical architecture or other cytologic abnormalities. Conclusion: Unexplained T2 hyperintensities occur frequently in patients with NF-2. While they may not be the NF-2 equivalent of NF-associated bright spots seen in NF-1, some of these T2 hyperintensities in patients with NF-2 may represent underlying disorders of neuronal migration. Further studies are needed to validate our findings.
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spelling doaj.art-e1abaceb31444f3385c1f6a1762e4be82022-12-22T01:33:39ZengElsevierNeuroImage: Clinical2213-15822014-01-014C25826510.1016/j.nicl.2013.12.010Incidental parenchymal magnetic resonance imaging findings in the brains of patients with neurofibromatosis type 2Wendy S. Vargas0Linda A. Heier1Fausto Rodriguez2Amanda Bergner3Kaleb Yohay4Division of Pediatric Neurology, Weill Cornell Medical Center/New York Presbyterian Hospital, USADivision of Neuroradiology, Weill Cornell Medical Center/New York Presbyterian Hospital, USADepartment of Pathology, Division of Neuropathology, Johns Hopkins University, USADepartment of Neurology, Johns Hopkins University, USADivision of Pediatric Neurology, Weill Cornell Medical Center/New York Presbyterian Hospital, USAPurpose: Whereas T2 hyperintensities known as NF-associated bright spots are well described in patients with neurofibromatosis type I (NF-1), there is a paucity of data on incidental findings in patients with neurofibromatosis type II (NF-2). We aim to characterize unexplained imaging findings in the brains of patients with NF-2. Materials and methods: This study is retrospective, HIPAA-compliant and approved by the institutional review board. 34 patients with NF-2 underwent brain magnetic resonance imaging (MRI) between January 2000 and December 2012. T2 and T1-weighted imaging characteristics, diffusion weighted imaging (DWI) characteristics, and enhancement patterns were analyzed by visual inspection. Clinical information at time of imaging was available for all patients. Neuropathologic data was available for one patient. Results: We found unexplained T2 hyperintensities present on initial imaging in 23/34 patients (67%). Of the 23 patients with unexplained MRI findings, 15 (65%) had wedge-shaped T2 hyperintensities in the subcortical white matter extending to the cortex suggestive of a cortical dysplasia. 3 additional cases (17%) had a lesion within the cerebellum suggestive of a neuronal migration anomaly. In one patient where the MRI was suggestive of focal cortical dysplasia, histopathologic analysis revealed dysplastic glial foci without other alterations of cortical architecture or other cytologic abnormalities. Conclusion: Unexplained T2 hyperintensities occur frequently in patients with NF-2. While they may not be the NF-2 equivalent of NF-associated bright spots seen in NF-1, some of these T2 hyperintensities in patients with NF-2 may represent underlying disorders of neuronal migration. Further studies are needed to validate our findings.http://www.sciencedirect.com/science/article/pii/S2213158213001691Neurofibromatosis type 2MRICortical dysplasiaMigration anomaliesNF-associated bright spotsMicro-hamartomas
spellingShingle Wendy S. Vargas
Linda A. Heier
Fausto Rodriguez
Amanda Bergner
Kaleb Yohay
Incidental parenchymal magnetic resonance imaging findings in the brains of patients with neurofibromatosis type 2
NeuroImage: Clinical
Neurofibromatosis type 2
MRI
Cortical dysplasia
Migration anomalies
NF-associated bright spots
Micro-hamartomas
title Incidental parenchymal magnetic resonance imaging findings in the brains of patients with neurofibromatosis type 2
title_full Incidental parenchymal magnetic resonance imaging findings in the brains of patients with neurofibromatosis type 2
title_fullStr Incidental parenchymal magnetic resonance imaging findings in the brains of patients with neurofibromatosis type 2
title_full_unstemmed Incidental parenchymal magnetic resonance imaging findings in the brains of patients with neurofibromatosis type 2
title_short Incidental parenchymal magnetic resonance imaging findings in the brains of patients with neurofibromatosis type 2
title_sort incidental parenchymal magnetic resonance imaging findings in the brains of patients with neurofibromatosis type 2
topic Neurofibromatosis type 2
MRI
Cortical dysplasia
Migration anomalies
NF-associated bright spots
Micro-hamartomas
url http://www.sciencedirect.com/science/article/pii/S2213158213001691
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