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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Autores principales: 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
Lenguaje:Spanish / Castilian
Publicado: Sociedad Médica de Santiago 2009
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Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872009000800011
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spelling 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
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