Síndrome moyamoya en paciente con síndrome de Down y déficit de antitrombina III
Moyamoya disease is a unique chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation. It can be primary or secondary to genetic syndromes such as Down syndrome. We report a...
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Sociedad Médica de Santiago
2009
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oai:scielo:S0034-988720090008000112009-11-04Síndrome moyamoya en paciente con síndrome de Down y déficit de antitrombina IIIHernández Ch,MartaHuete L,IsidroConcha G,María-JoséMéndez C,José ISánchez D,NicolásCuéllar G,María-CarolinaRetamal R,EvaMesa L,Tomás Antithrombin III deficiency Cerebrovascular Moyamoya disease Down syndrome Moyamoya disease is a unique chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation. It can be primary or secondary to genetic syndromes such as Down syndrome. We report a seven year-old girl with a Down syndrome that presented with a disturbance of consciousness, seizures and a right hemiparesia at the age of five. Magnetic resonance imaging showed old cortical ischemic lesions in both cerebral hemispheres and a recent infarction in the territory of the ¡eft middle cerebral artery. Brain angiography showed a proximal stenosis of both medial cerebral arteries and a net of collateral vessels, consistent with the diagnosis of moyamoya syndrome. The patient had also an antithrombin III deficiency. Aspirin was indicated and a surgical correction was recommended. However, prior to the procedure, the patient had a new infarction in the territory of the right middle cerebral artery, which caused a severe disability.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.137 n.8 20092009-08-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872009000800011es10.4067/S0034-98872009000800011 |
institution |
Scielo Chile |
collection |
Scielo Chile |
language |
Spanish / Castilian |
topic |
Antithrombin III deficiency Cerebrovascular Moyamoya disease Down syndrome |
spellingShingle |
Antithrombin III deficiency Cerebrovascular Moyamoya disease Down syndrome Hernández Ch,Marta Huete L,Isidro Concha G,María-José Méndez C,José I Sánchez D,Nicolás Cuéllar G,María-Carolina Retamal R,Eva Mesa L,Tomás Síndrome moyamoya en paciente con síndrome de Down y déficit de antitrombina III |
description |
Moyamoya disease is a unique chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation. It can be primary or secondary to genetic syndromes such as Down syndrome. We report a seven year-old girl with a Down syndrome that presented with a disturbance of consciousness, seizures and a right hemiparesia at the age of five. Magnetic resonance imaging showed old cortical ischemic lesions in both cerebral hemispheres and a recent infarction in the territory of the ¡eft middle cerebral artery. Brain angiography showed a proximal stenosis of both medial cerebral arteries and a net of collateral vessels, consistent with the diagnosis of moyamoya syndrome. The patient had also an antithrombin III deficiency. Aspirin was indicated and a surgical correction was recommended. However, prior to the procedure, the patient had a new infarction in the territory of the right middle cerebral artery, which caused a severe disability. |
author |
Hernández Ch,Marta Huete L,Isidro Concha G,María-José Méndez C,José I Sánchez D,Nicolás Cuéllar G,María-Carolina Retamal R,Eva Mesa L,Tomás |
author_facet |
Hernández Ch,Marta Huete L,Isidro Concha G,María-José Méndez C,José I Sánchez D,Nicolás Cuéllar G,María-Carolina Retamal R,Eva Mesa L,Tomás |
author_sort |
Hernández Ch,Marta |
title |
Síndrome moyamoya en paciente con síndrome de Down y déficit de antitrombina III |
title_short |
Síndrome moyamoya en paciente con síndrome de Down y déficit de antitrombina III |
title_full |
Síndrome moyamoya en paciente con síndrome de Down y déficit de antitrombina III |
title_fullStr |
Síndrome moyamoya en paciente con síndrome de Down y déficit de antitrombina III |
title_full_unstemmed |
Síndrome moyamoya en paciente con síndrome de Down y déficit de antitrombina III |
title_sort |
síndrome moyamoya en paciente con síndrome de down y déficit de antitrombina iii |
publisher |
Sociedad Médica de Santiago |
publishDate |
2009 |
url |
http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872009000800011 |
work_keys_str_mv |
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