Cerebral Vasculopathy in Children With Neurofibromatosis Type 1

Ghosh, Partha S.; Rothner, A. David; Emch, Todd; Friedman, Neil; Moodley, Manikum

doi:10.1177/0883073812441059

Cited by 53 publications

(35 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Dysplasia of the cerebral vasculature in children with NF-1 develops at a young age and most children are clinically asymptomatic. Prevalence in asymptomatic children (assessed with screening MRI exams) is up to 5% [5]. The incidence in the symptomatic NF-1 population appears to be higher, with a predicted range of 7-15% [20].…”

Section: Vascular Dysplasiamentioning

confidence: 97%

See 1 more Smart Citation

Neuroimaging of phakomatoses: overview and advances

Vézina

2015

Pediatr Radiol

View full text Add to dashboard Cite

The phakomatoses are disorders characterized by multiple hamartomas and other congenital malformations affecting mainly the skin and the central and peripheral nervous systems. Many affected individuals have an increased genetic susceptibility to develop malignancies. Imaging is central in the diagnosis of many of the phakomatoses, and MRI is used as a screening tool in many children with known neurocutaneous disorders. This manuscript addresses the three most common (neurofibromatosis type 1, tuberous sclerosis complex, Sturge-Weber syndrome) and focuses on pathophysiological and radiologic insights that have emerged in the last few years.

show abstract

Section: Vascular Dysplasiamentioning

confidence: 97%

“…(2) Regulation of neural stem cell proliferation, survival and astroglial differentiation, and neuroglial progenitor function [4]. (3) Maintenance of the vascular wall [5].…”

Section: Neurofibromatosis Typementioning

confidence: 99%

Neuroimaging of phakomatoses: overview and advances

Vézina

2015

Pediatr Radiol

View full text Add to dashboard Cite

show abstract

“…Neuroimaging may reveal low-grade gliomas mainly of the optic pathway, as well as within the brain stem and cerebellum 3 . In addition, cerebro-vasculopathies such as moyamoya syndrome may be observed 4 . One of the most intriguing NF1 lesions is the so-called "unidentified bright objects (UBOs) or neurofibromatosis bright objects (NBOs)" 5,6 .…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2014

View full text Add to dashboard Cite

SUMMARY -Neurofibromatosis bright objects (NBOs) are poorly understood. This article aimed to investigate: 1) differences in fractional anisotropy (FA) between NBOs based in gray matter (GM) and white matter (WM), and 2) the relationship between NBOs and the affected white matter tracts. Fourteen NF1 patients were included in this study. Apparent diffusion coefficient (ADC), FA, radial diffusivity (RD) and eigenvalues were used to compare NBOs and matching contralateral normalappearing sites (NAS). Diffusion tensor imaging scalars were also compared with age-matched healthy controls. Fiber tractography was performed to assess NBO-induced changes in WM trajectories. ADC values were higher for GM and WM NBOs than for NAS and controls. FA values were lower in GM and

show abstract

“…In the pediatric NF1 population, there is an increased prevalence of both peripheral and cerebral vasculopathy. 17,18 Finally, our recent work on pregnancy outcomes suggests that phenomena such as preeclampsia and cerebrovascular disease are more common in NF1. 19 Given the prevalence of vasculopathy and cerebrovascular anomalies with NF1, an elevated risk of stroke has been postulated in previous research.…”

mentioning

confidence: 99%

Increased Risk of Cerebrovascular Disease Among Patients With Neurofibromatosis Type 1

Terry

Jordan

Schwamm

et al. 2016

Stroke

View full text Add to dashboard Cite

Background and Purpose-Although neurofibromatosis type 1 (NF1) may be associated with an incompletely understood vasculopathy, relative odds of stroke in this population is not known. Methods-Using the 1998 to 2009 US Nationwide Inpatient Sample, we performed a case-control study matching cases of NF1 to controls without such a diagnosis. We then compared the odds of stroke between the 2 groups. We used multivariable logistic regression to adjust for known or suspected confounders. Results-NF1 was associated with younger mean age at the time of stroke (41 versus 48) and a lower prevalence of stroke risk factors among adult patients. Pediatric patients with NF1, however, were more likely to have hypertension. Patients with NF1 were significantly more likely to be diagnosed with any stroke (odds ratio, 1.2; P<0.0001) than the general population. The odds of intracerebral hemorrhage were greatest among hemorrhagic stroke types analyzed (odds ratio, 1.9; P<0.0001). In the pediatric NF1 population, the odds of intracerebral hemorrhage were more dramatically elevated (odds ratio, 8.1; P<0.0001). The odds of ischemic stroke were also increased with NF1 in the pediatric (odds ratio, 3.4; P<0.0001) but not in the adult population. Conclusions-When compared with the general population, the odds of any type of stroke are significantly increased for patients with NF1, both adult and pediatric. This risk is most notable for hemorrhagic strokes although it is also increased for ischemic strokes in children. Physicians should be aware of the increased risk of stroke in this population, and consider stroke as a potential cause of new neurological symptoms. (Stroke. 2016;47:60-65.

show abstract

Cerebral Vasculopathy in Children With Neurofibromatosis Type 1

Cited by 53 publications

References 21 publications

Neuroimaging of phakomatoses: overview and advances

Neuroimaging of phakomatoses: overview and advances

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

Increased Risk of Cerebrovascular Disease Among Patients With Neurofibromatosis Type 1

Contact Info

Product

Resources

About