Prominent myelin vacuolization in neurofibromatosis type 2

Şener, R. N.; Dzelzīte, Sarmīte; Migals, A.; Raits, U.; Veinbergs, A.A.

doi:10.1016/s0899-7071(02)00501-6

Cited by 5 publications

(4 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Histologically, hamartomatous lesions are composed of atypical glial infiltrates, foci of microcalcifications, perivascular gliosis and spongy peripheral changes [19]. Myelin vacuolization was also described in patients with NF II [20]. Vacuolar changes in the myelin or spongioform myelinopathy lead to high signal intensity on T2-w images.…”

Section: Ubosmentioning

confidence: 99%

Pediatric brain MRI in neurofibromatosis type I

et al. 2004

View full text Add to dashboard Cite

Neurofibromatosis (NF) is the most common of the phakomatoses, with a prevalence of 1 in 3-4,000. Many organ systems can be affected. In addition to multiple peripheral neurofibromas, NF I predisposed to CNS tumors including optic glioma, astrocytoma and plexiform neurofibroma. The purpose of this pictorial review is to illustrate characteristic brain MR imaging lesions in children with NF I and to give some recommendations about diagnostic imaging procedures in children suffering from NF I. Typical findings in brain MRI are hyperintense lesion on T2-weighted images, so-called unknown bright objects, which may be useful as an additional imaging criterion for NF I. Contrast administration is necessary in MR studies to maximize tumor detection and characterization, to add confidence to the diagnosis of benign probable myelin vacuolization, and to document stability of neoplasm on follow-up examinations. We recommend to perform serial MR imaging in children every 12 months. The frequency of follow-up in children with known brain tumors will vary with the tumor grade, biological activity and treatment.

show abstract

Section: Ubosmentioning

confidence: 99%

Pediatric brain MRI in neurofibromatosis type I

et al. 2004

View full text Add to dashboard Cite

show abstract

“…In addition to this finding, Mori et al [10] have reported slightly increases in ADC values within UBOs by serial examinations. Sener et al [12] reported high ADC values within the areas of myelin vacuolizations in a patient with NF2. Eastwood et al [13] have reported high ADC values within UBOs and normal appearing areas of the brain in children with NF1.…”

Section: Discussionmentioning

confidence: 97%

“…There are only a limited number of reports on the diffusion characteristics of UBOs [10][11][12][13][14]. In two of these studies, the authors found increased ADC values within UBOs [10,11].…”

Section: Discussionmentioning

confidence: 99%

Unidentified bright objects in neurofibromatosis type 1: Results of diffusion tensor imaging in children and adolescents

et al. 2015

View full text Add to dashboard Cite

To evaluate by means of diffusion tensor magnetic resonance imaging (DTI) if changes in apparent diffusion coefficient (ADC) and diffusion tensor fraction anisotropy (FA) in unidentified bright objects (UBOs) and perilesional normal appearing brain regions of children with neurofibromatosis type 1 (NF1) can be observed. Ten children with NF1 underwent conventional magnetic resonance imaging and DTI. The ADC and FA in UBOs and perilesional normal appearing brain of NF1 patients were compared with the same regions of the brain of controls. The ADC increased significantly for UBOs and surrounding normal appearing brain tissue of the brain in children with NF1. We observed no significant differences in FA between the UBOs and perilesional areas or that of controls. Although there is limited neuropathologic information about UBOs, detection of diffusion properties of UBOs can be used to understand tissue characteristics of UBOs, noninvasively.

show abstract

“…However, there is a lack of data on the existence and significance of unexplained imaging findings in the brains of patients with NF-2. One case report suggests that hyperintensities seen on T2-weighted imaging in the brain of a 21-year-old man with NF-2 were likely due to myelin vacuolization ( Sener et al, 2003 ). This assumption was based primarily on MRI appearance as there was no corresponding histopathology.…”

Section: Introductionmentioning

confidence: 99%

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

Vargas

Heier

Rodríguez

et al. 2014

NeuroImage: Clinical

View full text Add to dashboard Cite

PurposeWhereas 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 methodsThis 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.ResultsWe 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.ConclusionUnexplained 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.

show abstract

Prominent myelin vacuolization in neurofibromatosis type 2

Cited by 5 publications

References 6 publications

Pediatric brain MRI in neurofibromatosis type I

Pediatric brain MRI in neurofibromatosis type I

Unidentified bright objects in neurofibromatosis type 1: Results of diffusion tensor imaging in children and adolescents

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

Contact Info

Product

Resources

About