• 作者：Rogelio Dom&#237 ; nguez Moreno^a ; ^{rogelio_dm@hotmail.com} ; Karina Ordaz Ló ; pez^a ; José ; Luis Gutié ; rrez Morales^b ; Eduardo Ló ; pez Velá ; zquez^a ; Fausto Monobe Herná ; ndez^c ; V&#237 ; ctor Manuel Alor Jimé ; nez^a
• 关键词：Caró ; tidas ; Estenosis ; Moyamoya ; Proteí ; na C ; Sí ; ndrome de Down
• 刊名：Neurolog铆a Argentina
• 出版年：2012
• 期刊代码：pca377_18530028
• 类别：neu
• 出版时间：April-June, 2012
• 卷：4
• 期：2
• 页码：91-94
• 文件大小：498 K

Introduction

Moyamoya syndrome (MMS) is associated with neuroinfection, neurofibromatosis type 1, Down syndrome (DS), and so on. so the syndrome is more common than Moyamoya disease that's idiopathic, both entities present with stenosis of internal carotid arteries and branches with formation of collateral vessels, these patients develop strokes or cerebral hemorrhages. We report a case of MMS in a patient with DS and some of the most frequent associated disorders.

Case report

Male 19 years old was admitted because of acute right hemiparesis and headache, physical examination right hemibody strength 0/5 in the arm and 3/5 in the leg, with increased tendon reflexes, protein C deficiency and hyperthyroidism, CT of skull with frontoparietal infarction, Doppler shows bilateral internal carotid stenosis confirmed by angiotomography (beaded pattern) and foramen ovale in echocardiography, treated with a diuretic, citicoline and symptomatic drugs, good clinical evolution, discharged after 8 days internment in good condition.

Conclusions

MMS is three times more frequent in DS patients than in general population and is usually associated with deficiency of protein C and S, hyperthyroidism worsens stenosis. The treatment may be medical but in children is preferred bypass surgery by risk of new neurological events since otherwise the prognosis is poor. We recommend evaluate for MMS in all patients with DS and stroke.