Characteristics of Moyamoya Syndrome in Pediatric Patients With Neurofibromatosis Type 1

Brosius, Stephanie N.; Vossough, Arastoo; Fisher, Michael J.; Lang, Shih-Shan; Beslow, Lauren A.; George, Brandon; Ichord, Rebecca

doi:10.1016/j.pediatrneurol.2022.05.013

Cited by 10 publications

(5 citation statements)

References 55 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A systematic review was conducted to find literature on cases with neurofibromatosis type 1–associated Moyamoya syndrome. 232 At total of 152 literature cases of children ≤18 years of age (median age of moyamoya syndrome diagnosis, 6 years [IQR, 3–10.8 years]) were identified. Stroke or TIA was present at diagnosis in 46%.…”

Section: Stroke (Cerebrovascular Diseases)mentioning

confidence: 99%

2024 Heart Disease and Stroke Statistics: A Report of US and Global Data From the American Heart Association

Martin,

Aday,

Almarzooq

et al. 2024

Circulation

476

View full text Add to dashboard Cite

BACKGROUND: The American Heart Association (AHA), in conjunction with the National Institutes of Health, annually reports the most up-to-date statistics related to heart disease, stroke, and cardiovascular risk factors, including core health behaviors (smoking, physical activity, nutrition, sleep, and obesity) and health factors (cholesterol, blood pressure, glucose control, and metabolic syndrome) that contribute to cardiovascular health. The AHA Heart Disease and Stroke Statistical Update presents the latest data on a range of major clinical heart and circulatory disease conditions (including stroke, brain health, complications of pregnancy, kidney disease, congenital heart disease, rhythm disorders, sudden cardiac arrest, subclinical atherosclerosis, coronary heart disease, cardiomyopathy, heart failure, valvular disease, venous thromboembolism, and peripheral artery disease) and the associated outcomes (including quality of care, procedures, and economic costs). METHODS: The AHA, through its Epidemiology and Prevention Statistics Committee, continuously monitors and evaluates sources of data on heart disease and stroke in the United States and globally to provide the most current information available in the annual Statistical Update with review of published literature through the year before writing. The 2024 AHA Statistical Update is the product of a full year’s worth of effort in 2023 by dedicated volunteer clinicians and scientists, committed government professionals, and AHA staff members. The AHA strives to further understand and help heal health problems inflicted by structural racism, a public health crisis that can significantly damage physical and mental health and perpetuate disparities in access to health care, education, income, housing, and several other factors vital to healthy lives. This year’s edition includes additional global data, as well as data on the monitoring and benefits of cardiovascular health in the population, with an enhanced focus on health equity across several key domains. RESULTS: Each of the chapters in the Statistical Update focuses on a different topic related to heart disease and stroke statistics. CONCLUSIONS: The Statistical Update represents a critical resource for the lay public, policymakers, media professionals, clinicians, health care administrators, researchers, health advocates, and others seeking the best available data on these factors and conditions.

show abstract

Section: Stroke (Cerebrovascular Diseases)mentioning

confidence: 99%

2024 Heart Disease and Stroke Statistics: A Report of US and Global Data From the American Heart Association

Martin,

Aday,

Almarzooq

et al. 2024

Circulation

476

View full text Add to dashboard Cite

show abstract

“…In the brain, the most commonly recognized abnormalities include moyamoya syndrome, stenosis, occlusion, hypoplasia, fusiform dilation, ectasia, aneurysm and arteriovenous fistulae (AVF). Estimates of prevalence rate in patients with NF1 range from 2.3 to 7.4% ascertained from retrospective cohort studies and case series [3][4][5][6][7][8][9][10][11][12]. Cerebral aneurysms have been reported in association with NF1 in cohort studies and case series [10,11]; however, a retrospective autopsy study did not find any association between NF1 and cerebral aneurysms [13].…”

Section: Pathogenesismentioning

confidence: 99%

“…Ghosh et al [7] similarly reported that optic pathway gliomas on imaging were present in 50% of patients with cerebral arteriopathy. Lastly, in a recent large cohort of children with NF1, 13 of 15 (87%) who had undergone surveillance neuroimaging specifically for screening of optic pathway glioma had moyamoya; vascular changes were found on initial imaging in 31% [3].…”

Section: Pathogenesismentioning

confidence: 99%

“…There is lack of consensus as to the association of CVD and causality due to other manifestations of NF1. Moyamoya can occur in individuals with or without NF1, although there is clearly an increased risk to those with NF1 [3,14,19,20]. Moyamoya is also the most frequently observed cerebrovascular abnormality in NF1 and is characterized by progressive stenosis of the internal carotid artery as well as stenosis of the other major intracranial arteries affecting anterior greater than posterior circulation.…”

Section: Pathogenesismentioning

confidence: 99%

“…Children with NF1 may undergo more frequent imaging due to screening for and follow-up of optic pathway glioma, which in turn may lead to detection of otherwise asymptomatic vascular changes and/or an earlier diagnosis of CVD. Despite this association, it is not clear if this the relationship is causal or if it is unrelated and is confounded by the increased frequency of imaging [3,9]. Mechanistically, it has been postulated that the presence of a tumor may release growth factors, which in turn impact the vascular endothelial proliferation [21].…”

Section: Pathogenesismentioning

confidence: 99%

See 2 more Smart Citations

Cerebral Vasculopathy in Children with Neurofibromatosis Type 1

Lehman,

Ullrich

2023

Cancers

View full text Add to dashboard Cite

Cerebrovascular abnormalities are a severe and often underrecognized complication of childhood neurofibromatosis type 1 (NF1). There are no prospective studies of cerebral vasculopathy in NF1; thus, the estimated frequency of vasculopathy varies between studies. The data is difficult to interpret due to the retrospective data collection and variability in whether imaging is done based on screening/surveillance or due to acute neurologic symptoms. The prevalent NF1-associated cerebral vasculopathy is moyamoya syndrome (MMS). Vascular changes can present without symptoms or with acute TIA or stroke-like symptoms or a range of progressive neurologic deficits. Advanced imaging may enhance sensitivity of neuroimaging in children. Medical and/or surgical interventions may prevent short- and long-term complications. Challenges for establishment of a screening protocol for cerebral vasculopathy in children with NF1 include the relatively large number of patients with NF1, the potential need for sedation to achieve quality imaging and the broad age range at time of detection for cerebral vascular changes. The goal of this review is to present the epidemiology, clinical presentation, imaging features and medical/surgical management of cerebral arteriopathies in children with NF1.

show abstract

Low-grade glioma in children with neurofibromatosis type 1: surveillance, treatment indications, management, and future directions

Kotch,

de Blank,

Gutmann

et al. 2024

Childs Nerv Syst

View full text Add to dashboard Cite

Characteristics of Moyamoya Syndrome in Pediatric Patients With Neurofibromatosis Type 1

Cited by 10 publications

References 55 publications

2024 Heart Disease and Stroke Statistics: A Report of US and Global Data From the American Heart Association

2024 Heart Disease and Stroke Statistics: A Report of US and Global Data From the American Heart Association

Cerebral Vasculopathy in Children with Neurofibromatosis Type 1

Low-grade glioma in children with neurofibromatosis type 1: surveillance, treatment indications, management, and future directions

Contact Info

Product

Resources

About