Prothrombotic Disorders in Children With Moyamoya Syndrome

Bonduel, Mariana; Hepner, Mirta; Sciuccati, Gabriela; Torres, Aurora Feliú; Tenembaum, Sílvia

doi:10.1161/01.str.32.8.1786

Cited by 74 publications

(67 citation statements)

References 62 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…35 A study from 2002 to 2006 states the incidence rate is now up to 0.94 patients per 100,000 people, with a prevalence of 10.5 patients per 100,000. 5 Moyamoya disease in this survey was more prevalent in women than men, with a female to male ratio of 1.8:1. 35 The survey showed the highest prevalence for males at ages [10][11][12][13][14] and smaller peaks at 35-39 and 55-59.…”

Section: Epidemiologymentioning

confidence: 63%

“…57 Protein S deficiency, lupus anticoagulant, and anticardiolipin antibodies have been found in other cases. 5 Anticardiolipin antibody binds to phosphatidyl-glycerol, a component of the plasma membrane. Its higher concentration in MMD patients suggests a possible autoimmune mechanism in MMD as the antibody could contribute to thrombus formation in strokes.…”

Section: Pathophysiologymentioning

confidence: 99%

See 1 more Smart Citation

Moyamoya disease: a summary

Burke

Burke²,

Sherma

et al. 2009

FOC

185

150

View full text Add to dashboard Cite

Moyamoya, meaning a “hazy puff of smoke” in Japanese, is a chronic, occlusive cerebrovascular disease involving bilateral stenosis or occlusion of the terminal portion of the internal carotid arteries (ICAs) and/or the proximal portions of the anterior cerebral arteries and middle cerebral arteries (MCAs). The Ministry of Health and Welfare of Japan has defined 4 types of moyamoya disease (MMD): ischemic, hemorrhagic, epileptic, and “other.” The ischemic type has been shown to predominate in childhood, while the hemorrhagic type is more often observed in the adult population. The highest prevalence of MMD is found in Japan, with a higher female to male ratio. Studies have shown a possible genetic association of MMD linked to chromosome 17 in Japanese cases as well as in cases found in other demographics. During autopsy, intracerebral hematoma is found and most commonly serves as the major cause of death in patients with MMD. Moyamoya vessels at the base of the brain are composed of medium-sized or small muscular arteries emanating from the circle of Willis, mainly the intracranial portions of ICAs, anterior choroidal arteries, and posterior cerebral arteries, forming complex channels that connect with distal positions of the MCAs. Off of these channels are small tortuous and dilated vessels that penetrate into the base of the brain at the site of the thalamoperforate and lenticulostriate arteries. On angiography, there is the characteristic stenosis or occlusion bilaterally at the terminal portion of the ICAs as well as the moyamoya vessels at the base of the brain. Six angiographic stages have been described, from Stage 1, which reveals a narrowing of the carotid forks, to Stage 6, in which the moyamoya vessels disappear and collateral circulation is produced solely from the external carotid arteries. Cases with milder symptoms are usually treated conservatively; however, more severe symptomatic cases are treated using revascularization procedures. Surgical treatments are divided into 3 types: direct, indirect, and combined/other methods. Direct bypass includes superficial temporal artery-MCA bypass or use of other graft types. Indirect procedures bring in circulation to the intracranial regions by introducing newly developed vasculature from newly approximated tissues. These procedures may not be enough to prevent further ischemia; therefore, a combination of direct and indirect procedures is more suitable. This article will give a review of the epidemiology, natural history, pathology, pathophysiology, and diagnostic criteria, including imaging, and briefly describe the surgical treatment of MMD.

show abstract

Section: Epidemiologymentioning

confidence: 63%

Section: Pathophysiologymentioning

confidence: 99%

Moyamoya disease: a summary

Burke

Burke²,

Sherma

et al. 2009

FOC

185

150

View full text Add to dashboard Cite

show abstract

“…Bonduel et al (5) reported that prothrombotic disorders were detected in 4 out of 10 non-Japanese children with Moyamoya. In their series, inherited PSD was found in 1 patient; LA and ACA were detected in the remaining 3 patients.…”

Section: Discussionmentioning

confidence: 99%

“…Oral anticoagulation is recommended if there is isolated PSD because of the possibility of a recurrent thrombosis. However, prophylactic oral anticoagulation is controversial in MMS patients with PSD due to the risk of intracranial haemorrhage (5,6). Because of PIVH, we postponed anti-coagulation in the acute stage.…”

Section: Discussionmentioning

confidence: 99%

Protein S Deficiency and an Adult Case with Moyamoya Syndrome that Presented with Primary Intraventricular Haemorrhage

Çevik¹,

Acu²,

Aksoy³

et al. 2014

Balkan Med J

View full text Add to dashboard Cite

“…10,14) Moyamoya syndrome may include inherited disorders such as Down syndrome, 5) neurofibromatosis 1, 9) and protein C deficiency, 7) as well as other acquired pathological conditions which may be involved in the mechanisms causing such moyamoya phenomena. [1][2][3] Autoimmune mechanisms such as Grave's disease, 3) Behcet's disease, 6) and antiphospholipid syndrome 1) may also be involved in the development of moyamoya syndrome, although cross-reactivity of autoantibodies to cerebral vessels has not yet been clearly demonstrated. Genetic factors may also contribute as the possible locations of genes for familial moyamoya disease have been demonstrated.…”

Section: Introductionmentioning

confidence: 99%

Coexistence of Chiari 2 Malformation and Moyamoya Syndrome in a 17-Year-Old Girl -Case Report-

Suyama

Yoshida

Hayashi

et al. 2009

Neurol. Med. Chir.(Tokyo)

View full text Add to dashboard Cite

A 17-year-old female with Chiari 2 malformation developed cerebral infarction with angiographically typical bilateral moyamoya vessels manifesting as sudden onset of moderate left hemiparesis. Magnetic resonance imaging revealed multiple infarcts in the right frontal lobe, agenesis of the corpus callosum, upward herniation of the dorsal cerebellum, tectal beak of the midbrain, and downward herniation of the cerebellar vermis. Cerebral angiography demonstrated occlusion of the bilateral internal carotid arteries and basal moyamoya vessels. Single photon emission computed tomography showed significantly reduced regional cerebral blood flow in the right frontoparietal cortex. The cerebral vascular reactivity to acetazolamide was diminished in both cerebral hemispheres. She underwent superficial temporal artery-middle cerebral artery anastomosis combined with encephalo-myo-synangiosis on the right, and on the left 6 months later. Cerebral angiography performed 4 months after the second operation showed good patency of the bypasses and substantial collateral vessels in both cerebral hemispheres. This association may have happened by chance, and a common etiology is uncertain, but a currently undetermined genomic component might have contributed to the disease progression.

show abstract

Prothrombotic Disorders in Children With Moyamoya Syndrome

Cited by 74 publications

References 62 publications

Moyamoya disease: a summary

Moyamoya disease: a summary

Protein S Deficiency and an Adult Case with Moyamoya Syndrome that Presented with Primary Intraventricular Haemorrhage

Coexistence of Chiari 2 Malformation and Moyamoya Syndrome in a 17-Year-Old Girl -Case Report-

Contact Info

Product

Resources

About