LGG-03. EFFECT OF AGE AND NEUROFIBROMATOSIS TYPE 1 STATUS ON WHITE MATTER INTEGRITY IN THE OPTIC RADIATIONS IN CHILDREN

Abstract Background Adults with neurofibromatosis type 1 (NF1) have decreased white matter integrity, but differences in children with NF1 have not been described. Defining normal values for diffusion tensor imaging (DTI) measures, especially in the optic radiations, is important to the development of DTI as a potential biomarker of visual acuity in children with optic pathway glioma. This study examines the effect of age and NF1 status on DTI measures in children. Methods In this retrospective study, MR imaging including DTI was conducted in 93 children (40 children with NF1 and 53 healthy controls) between 0 and 14 years of age. Regression models of age, sex, and NF1 status on DTI measures were evaluated, and tract-based spatial statistics (TBSS) compared DTI measures in age-matched NF1 to non-NF1 cohorts. Results Fractional anisotropy, radial diffusivity, and mean diffusivity in white matter tracts of the optic radiations varied with age and were best modeled by a logarithmic function. Age-related DTI measure change was different in NF1 versus non-NF1 subjects. Normal values and 95% confidence intervals for age 0.5–12 years were derived for both groups. Differences in DTI measures between NF1 and non-NF1 groups at a range of ages were shown diffusely throughout the cerebral white matter using TBSS. Conclusions Children with NF1 demonstrate increased diffusion throughout the brain compared to children without NF1 suggesting a potentially altered developmental trajectory of optic radiation microstructure. Defining normal values for white matter integrity in children with NF1 may help target early intervention efforts in this vulnerable group.

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Systemic Chemotherapy and White Matter Integrity in Tracts Associated with Cognition Among Children With Neurofibromatosis Type 1

Pediatric Blood & Cancer ◽

10.1002/pbc.25896 ◽

2016 ◽

Vol 63 (5) ◽

pp. 818-824 ◽

Cited By ~ 10

Author(s):

Peter Matthew Kennedy de Blank ◽

Jeffrey I. Berman ◽

Michael Jay Fisher

Keyword(s):

White Matter ◽

Neurofibromatosis Type 1 ◽

Systemic Chemotherapy ◽

Neurofibromatosis Type ◽

White Matter Integrity

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Fractional anisotropy of the optic radiations is associated with visual acuity loss in optic pathway gliomas of neurofibromatosis type 1

Neuro-Oncology ◽

10.1093/neuonc/not068 ◽

2013 ◽

Vol 15 (8) ◽

pp. 1088-1095 ◽

Cited By ~ 29

Author(s):

P. M. K. de Blank ◽

J. I. Berman ◽

G. T. Liu ◽

T. P. L. Roberts ◽

M. J. Fisher

Keyword(s):

Visual Acuity ◽

Fractional Anisotropy ◽

Neurofibromatosis Type 1 ◽

Neurofibromatosis Type ◽

Optic Pathway ◽

Visual Acuity Loss ◽

Optic Radiations ◽

Optic Pathway Gliomas

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Brain white matter structures in Learning Disabilities in Neurofibromatosis type 1

International Journal of Psychophysiology ◽

10.1016/j.ijpsycho.2016.07.493 ◽

2016 ◽

Vol 108 ◽

pp. 172

Author(s):

Nadir Díaz-Simón ◽

Nancy Estévez-Pérez ◽

Daylín Góngora-Lleonart ◽

Maydel Fernandez-Alonso ◽

Vivian Reigosa-Crespo ◽

...

Keyword(s):

Learning Disabilities ◽

White Matter ◽

Neurofibromatosis Type 1 ◽

Neurofibromatosis Type ◽

Brain White Matter

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NFB-04. EXAMINING DIFFUSION, ARTERIAL SPIN-LABELED PERFUSION, AND VOLUMETRIC CHANGES IN THE NEUROFIBROMATOSIS TYPE 1 BRAIN USING AN ATLAS-BASED, MULTI-PARAMETRIC APPROACH

Neuro-Oncology ◽

10.1093/neuonc/noaa222.608 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii418-iii418

Author(s):

Lydia Tam ◽

Nathan Ng ◽

Peter Moon ◽

Jimmy Zheng ◽

Emily McKenna ◽

...

Keyword(s):

Blood Flow ◽

Cerebral Blood Flow ◽

White Matter ◽

Neural Development ◽

Neurofibromatosis Type 1 ◽

Neurofibromatosis Type ◽

Analysis Of Covariance ◽

Brain Atlas ◽

Parametric Approach

Abstract BACKGROUND Neurofibromatosis Type 1 (NF1) is a multisystem disorder with wide ranging clinical implications. Patients may present with macrocephaly, stroke, and cognitive deficits, all of which may impede normal neural development. We applied atlas-based, multi-parametric MRI analysis of regional brain to evaluate diffusion, arterial spin-labeled (ASL) perfusion, and volumetric changes in children with NF1. METHODS Children evaluated for NF1 from 2009 to 2018 at Stanford University (n=78) were retrospectively reviewed and compared to healthy controls (n=100). All patients underwent diffusion-weighted (DWI) magnetic resonance imaging at 3T, and children with brain tumors were excluded. Using atlas-based DWI analyses, we assessed volume, median apparent diffusion coefficient (ADC), and cerebral blood flow in the cerebral cortex, thalamus, caudate, putamen, globus pallidus, hippocampus, amygdala, nucleus accumbens, brain stem, and cerebral white matter. We also measured volume of the lateral ventricles. Multivariate analysis of covariance was used to test for differences between controls and NF1 patients, controlling for gender and age at time of imaging. RESULTS Comparing NF1 to controls, we detected increased volume and decreased ASL cerebral blood flow in white matter and all subcortical and cortical structures except for brainstem volume. Median ADC was also increased in the thalamus, pallidum, hippocampus, and brainstem. CONCLUSIONS Using a multi-parametric approach, we demonstrate quantitative measures of microstructural and physiologic changes of the NF1 brain. Atlas-based, quantitative MRI brain signatures may serve as biomarkers of neural development and further provide insight into associated cognitive dysfunction or risks for vasculopathy-related strokes in children with NF1.

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White matter microstructure of patients with neurofibromatosis type 1 and its relation to inhibitory control

Brain Imaging and Behavior ◽

10.1007/s11682-016-9641-3 ◽

2016 ◽

Vol 11 (6) ◽

pp. 1731-1740 ◽

Cited By ~ 15

Author(s):

M. Koini ◽

S. A. R. B. Rombouts ◽

I. M. Veer ◽

M. A. Van Buchem ◽

S. C. J. Huijbregts

Keyword(s):

White Matter ◽

Inhibitory Control ◽

Neurofibromatosis Type 1 ◽

Neurofibromatosis Type ◽

White Matter Microstructure

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I foci iperintensi in corso di neurofibromatosi hanno sempre una evoluzione benigna?

Rivista di Neuroradiologia ◽

10.1177/19714009970100s2107 ◽

1997 ◽

Vol 10 (2_suppl) ◽

pp. 236-239

Author(s):

P.L. Lanza ◽

W. Auteri ◽

A. Armentano ◽

G. De Vuono ◽

G. Santoro

Keyword(s):

White Matter ◽

Neurofibromatosis Type 1 ◽

Normal Tissue ◽

Neurofibromatosis Type ◽

Low Grade ◽

True Nature ◽

Periventricular Lesion ◽

Mr Brain ◽

Mr Characteristics

More than 70% of patients with neurofibromatosis type 1 (NF-1), present hyperintense foci in MR DP-T2 weighted images, located in deep grey and white matter. The true nature of these hyperintense foci is still unknown: they could be hamartomas, that is composition and organisation alterated areas of cytologic normal tissue, gliotic areas, delayed or disorganised areas of myelinisation and, finally, low grade glial tumors (astrocytoma). We present the case of a 17 years old pazient, who underwent 8 MR brain studies in a six years period. We analyzed the MR characteristics of the hyperintense foci in seriated studies and, particularly, the evolution of a single, simple, hyperintense periventricular lesion in cistic tumor with intramural nodule.

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Effects of Age on White Matter Microstructure in Children With Neurofibromatosis Type 1

Journal of Child Neurology ◽

10.1177/08830738211008736 ◽

2021 ◽

pp. 088307382110087

Author(s):

Lydia T. Tam ◽

Nathan N. Ng ◽

Emily S. McKenna ◽

Lisa Bruckert ◽

Kristen W. Yeom ◽

...

Keyword(s):

Early Childhood ◽

White Matter ◽

Corpus Callosum ◽

Fractional Anisotropy ◽

Middle Childhood ◽

Neurofibromatosis Type 1 ◽

Neurofibromatosis Type ◽

Childhood And Adolescence ◽

White Matter Microstructure

Children with neurofibromatosis type 1 (NF1) often report cognitive challenges, though the etiology of such remains an area of active investigation. With the advent of treatments that may affect white matter microstructure, understanding the effects of age on white matter aberrancies in NF1 becomes crucial in determining the timing of such therapeutic interventions. A cross-sectional study was performed with diffusion tensor imaging from 18 NF1 children and 26 age-matched controls. Fractional anisotropy was determined by region of interest analyses for both groups over the corpus callosum, cingulate, and bilateral frontal and temporal white matter regions. Two-way analyses of variance were done with both ages combined and age-stratified into early childhood, middle childhood, and adolescence. Significant differences in fractional anisotropy between NF1 and controls were seen in the corpus callosum and frontal white matter regions when ages were combined. When stratified by age, we found that this difference was largely driven by the early childhood (1-5.9 years) and middle childhood (6-11.9 years) age groups, whereas no significant differences were appreciable in the adolescence age group (12-18 years). This study demonstrates age-related effects on white matter microstructure disorganization in NF1, suggesting that the appropriate timing of therapeutic intervention may be in early childhood.

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