Moyamoya disease and protein S deficiency: A case report

Charuvanij, Araya; Laothamatas, Jiraporn; Torcharus, Kitti; Sirivimonmas, Somkiet

doi:10.1016/s0887-8994(97)00072-6

Cited by 13 publications

(8 citation statements)

References 15 publications

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“…39 Few reports have described the probable relationship between hemostatic abnormalities and MMS. [15][16][17][18][19][20][21][22][23][24][25] In our series, a prothrombotic disorder was detected in 4 of our patients (40%). Inherited protein S deficiency was detected in 1 patient, and persistent positive LA or ACA was found for months or even years in the remaining 3 patients.…”

Section: Discussionmentioning

confidence: 85%

“…[2][3][4][5][6][7][8][9][10][11][12][13][14] The contribution of prothrombotic disorders to the pathogenesis of MMS in children is still to be elucidated because available data are related only to retrospective studies of few patients. [15][16][17][18][19][20][21][22][23][24][25] The main aim of this work was to perform a prospective study, including a complete hemostatic evaluation, to determine the frequency of prothrombotic disorders in a pediatric population with MMS.…”

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confidence: 99%

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Prothrombotic Disorders in Children With Moyamoya Syndrome

Bonduel¹,

Hepner

Sciuccati

et al. 2001

Stroke

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Background and Purpose — Moyamoya syndrome is an uncommon chronic occlusive cerebrovascular disease in children. The origin of moyamoya syndrome remains undetermined. The role of the prothrombotic disorders contributing to its pathogenesis has not been completely elucidated. The purpose of this study was to determine the frequency of prothrombotic disorders in a pediatric population with moyamoya syndrome. Methods — From May 1992 to April 2000, a prospective study of 10 consecutive children with moyamoya syndrome was carried out at a single center. Evaluation included the following assays: protein C, protein S, antithrombin, plasminogen, activated protein C resistance, factor V Leiden, and prothrombin gene mutations. Lupus anticoagulant, anticardiolipin antibodies, and anti–β 2 -glycoprotein I antibodies assays were also performed. The clinical characteristics, underlying diseases, family history of thrombosis, radiological findings, treatment, and outcome were also recorded. Results — In our series, prothrombotic disorders were detected in 4 patients (40%). Inherited protein S deficiency was found in 1 patient; lupus anticoagulant and anticardiolipin antibodies were detected in the remaining 3 patients. One presented persistent lupus anticoagulant for 2.7 years until his death. In the case of the other 2 patients, 1 has maintained lupus anticoagulant for 9 months, whereas the other has kept anticardiolipin/anti–β 2 -glycoprotein I antibodies for 10 months. Conclusions — We report the hemostatic data of the largest prospective pediatric study carried out at a single center in the western hemisphere. In 4 patients (40%), a prothrombotic disorder was detected. It is tempting to speculate that these hemostatic abnormalities may contribute to the pathogenesis of moyamoya syndrome in some of our patients.

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Section: Discussionmentioning

confidence: 85%

mentioning

confidence: 99%

Prothrombotic Disorders in Children With Moyamoya Syndrome

Bonduel¹,

Hepner

Sciuccati

et al. 2001

Stroke

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“…1,3) In our series, the serum level of protein C did not decrease to the significant level of congenital protein S deficiency, which was around 20%. [1][2][3] Although C3-positive granules were observed on the endoplasmic reticulum of endothelial and intimal cells of MMD patients, 16) the relationship between increased complement C3 in serum and MMD has also not been clarified. Change in the milieu inside the vessels may contribute to these kinds of prothrombotic conditions.…”

Section: J E Kim Et Almentioning

confidence: 41%

Clinical Features of Adult Moyamoya Disease With Special Reference to the Diagnosis

Kim

Kang

et al. 2012

Neurol. Med. Chir.(Tokyo)

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Fifteen years of experience with adult moyamoya disease (MMD) in a single department were analyzed to describe the clinical features and to clarify the existence of the disease entity of adult MMD with special reference to its diagnosis. This study included 348 patients treated between 1996 and 2010. Male to female ratio was 128 to 220 with mean age of 39 years. The presence of neurological symptoms and signs during childhood and mean Suzuki stage according to the age were evaluated. Adult MMD was differentiated from other diseases, such as vasculitis, by comparing the laboratory data including common markers and specific markers for such diseases between 22 adult MMD and 21 atherosclerotic disease (ASD) patients. Conventional angiographic data were analyzed to detect progression in 52 unilateral adult MMD patients over a mean follow-up duration of 4.8 years. Only one of 128 patients (0.8%) aged over 40 years showed childhood symptoms and signs. The distribution of the mean Suzuki stage between patients aged 40 years and under and over 40 years was similar. Laboratory tests differentiating the other diseases with the same phenotype with MMD revealed no difference between adult MMD and ASD. The progression of unilateral MMD to bilateral MMD occurred in 6 of 52 patients (11.5%). Adult MMD is not a syndrome, but a distinct disease entity with significant progression of unilateral MMD to bilateral MMD. Diagnostic and therapeutic strategies should be different from pediatric cases when considering adult MMD.

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“…Çeşitli yayınlarda moyamoya hastalığı ile Down sendromu, orak hücreli anemi, nörofibromatoz ve tüberküloz menenjiti gibi hastalıkların birlikteliği bildirilmiştir. [11][12][13][14] Bu duruma hastalık yerine "moyamoya sendromu" denilmektedir. Rapor edilen hastaların %7-12'sinde ailede moyamoya hastalığı öyküsü saptanmıştır.…”

Section: Discussionunclassified