This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Bonduel, M. Articles by de Veber, G. Search for Related Content PubMed PubMed Citation Articles by Bonduel, M. Articles by de Veber, G. Related Collections Thrombophilia

(Stroke. 2001;32:1786.)
© 2001 American Heart Association, Inc.

Original Contributions

Prothrombotic Disorders in Children With Moyamoya Syndrome

Mariana Bonduel, MD; Mirta Hepner, PhD; Gabriela Sciuccati, MD; Aurora Feliú Torres, MD Silvia Tenembaum, MD

From Servicio de Hematología-Oncología, Hospital de Pediatría "Prof Dr Juan P. Garrahan," Buenos Aires, Argentina.

Correspondence to Mariana Bonduel, MD, Servicio de Hematología-Oncología, Hospital de Pediatría "Prof Dr Juan P. Garrahan," Combate de los Pozos 1881, C1245AAM Buenos Aires, Argentina.

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–ß₂-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–ß₂-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.

Editorial Comment

Gabrielle de Veber, MD; Guest Editor

Division of Neurology, Hospital for Sick Children, Toronto, Ontario, Canada

This article has been cited by other articles:

				H. Jeong, Y. Kim, W Yoon, S. Joo, S. Lee, and Y. Park Moyamoya syndrome associated with systemic lupus erythematosus Lupus, July 1, 2008; 17(7): 679 - 682. [Abstract] [PDF]

				S. Gokben, A. Tosun, N. Bayram, G. Serdaroglu, M. Polat, K. Kavakli, and H. Tekgul Arterial Ischemic Stroke in Childhood: Risk Factors and Outcome in Old Versus New Era J Child Neurol, October 1, 2007; 22(10): 1204 - 1208. [Abstract] [PDF]

				A. Skardoutsou, K. A. Voudris, S. Mastroyianni, E. Vagiakou, G. Magoufis, and P. Koukoutsakis Moya Moya Syndrome in a Child With Pyruvate Kinase Deficiency and Combined Prothrombotic Factors J Child Neurol, April 1, 2007; 22(4): 474 - 478. [Abstract] [PDF]

				L. I. Kornblihtt, S. Cocorullo, C. Miranda, P. Lylyk, P. G. Heller, and F. C. Molinas Moyamoya Syndrome in an Adolescent With Essential Thrombocythemia: Successful Intracranial Carotid Stent Placement Stroke, August 1, 2005; 36(8): e71 - e73. [Abstract] [Full Text] [PDF]

				T.-H. Lee, W.-C. Hsu, C.-J. Chen, and S.-T. Chen Etiologic Study of Young Ischemic Stroke in Taiwan Stroke, August 1, 2002; 33(8): 1950 - 1955. [Abstract] [Full Text] [PDF]