Quantitative Differentiation Between Healthy and Disordered Brain Matter in Patients with Neurofibromatosis Type I Using Diffusion Tensor Imaging

Abstract

BACKGROUND AND PURPOSE:Hyperintensities on T2-weighted images are seen in the brains of most patients with neurofibromatosis type I (NF-1), but the origin of these unidentified bright objects (UBOs) remains obscure. In the current study, we examined the diffusion characteristics of brain tissue in children with NF-1 to test the hypothesis that a microstructural abnormality is present in NF-1.

Corpus Callosum Morphology and Microstructure Assessed Using Structural MR Imaging and Diffusion Tensor Imaging: Initial Findings in Adults with Neurofibromatosis Type 1

“…The first, van Engelen et al, 20 did not report the findings of the eigenvalues; the latter, Zamboni et al, 21 did, reporting falls in all 1 , 2, and 3. It has been postulated in the Zamboni study of pediatric patients with NF-1 on the basis of all 3 eigenvalues being reduced that the primary cause for the reduced FA was related to vacuolation or edema of the myelin, as has been found on pathologic study of the brain.…”

“…20,21 There is broad correlation between the area of the CC and total brain volume, though the relationship between regional assessment of CC size and whole-brain volume in controls is not clearly established. It appears to depend on the technique of assessing the whole-brain size (wet weight, midsagittal area, forebrain volume) and the method of CC measurement used.…”

“…26,27 One possible explanation for the increased size of the CC in children with NF-1 is a delay and/or reduction in apoptosis, 3,6 which would produce greater numbers of commissural fibers. Other possible causes include larger axons due to excessive myelination, increased extracellular fluid, myelinopathy associated with vacuolation, 20,28 or a combination of those factors. Moore et al 6 found that differences in gray matter volume were more pronounced in younger subjects with NF-1, which led them to suggest that apoptosis was delayed rather than absent.…”

Diffusion Tensor Imaging of Neurofibromatosis Bright Objects in Children with Neurofibromatosis Type 1

“…The small number of scientific papers evaluating NBOs by diffusion-weighted imaging have mostly focused on studying the appar-ent diffusion coefficient (ADC) values of NBOs [11][12][13][14] . Few studies have evaluated the fractional anisotropy (FA) values and the corresponding eigenvalues of these lesions [15][16][17][18][19] . In addition, no study is available that systematically applied fiber tractography (FT) to the evaluation of NBOs and their relation to WM tracts within the brain.…”

Diffusion Tensor Imaging and Fiber Tractography in Children with Craniosynostosis Syndromes

“…In addition, we included 7 healthy control subjects within the same age range who were previously neuropsychologically tested and scanned (identical scanner and MR imaging protocol) for another study. 18 Exclusion criteria for all subjects were the following: insufficient quality of the collected DTI dataset due to incomplete scanning, motion artifacts, or inhomogeneity of the MR imaging field due to braces or metallic remains from operations. In total, we excluded 7 patients with craniosynostosis syndromes: 1 had Apert syndrome, 1 had Crouzon-Pfeiffer syndrome, 2 had Muenke syndrome, 1 had Saethre-Chotzen syndrome, and 2 had complex craniosynostosis.…”