conclusions in cases of unusual vascular lesions, metabolic diseases must be considered. in homocystinuria, early diagnosis and treatment are important. blood homocysteine levels can be returned to normal, and some complications can be prevented. introduction homocystinuria is a hereditary disease caused by a defect in the enzymes involved in metabolizing methionine. homocystinuria can influenc...

case presentation we reported a 2-year-old boy with pph, who presented unusual and intractable seizure which suggested the diagnosis of md. we also reviewed a few previous reports about the association of pph with moyamoya disease. discussion moyamoya disease is a kind of cerebral vasculopathy and should be considered as a differential diagnosis for patients with unusual seizure attacks who are...

This study investigated the incidences of persistent primitive arteries in patients with moyamoya disease, unilateral moyamoya disease, and quasi-moyamoya disease. Cerebral angiograms of 50 patients (39 moyamoya disease patients, 6 unilateral moyamoya disease patients, and 5 quasi-moyamoya disease patients) were retrospectively reviewed. There were 35 females and 15 males, aged from 3 to 63 yea...

Moyamoya disease is a rare vaso-occlusive illness with an unknown etiology characterized by stenosis of the internal carotid arteries with spontaneous development of a collateral vascular network.A 15-month-old girl was referred to the emergency ward of Imam Reza Hospital due to decreased level of consciousness, focal seizures and fever during the previous 24 hours with an impression of encepha...

Moyamoya disease is characterized by progressive occlusion of the internal carotid artery or its terminal branches, associated with formation of extensive collateral vessels (moyamoya vessels) at the base of the brain. Whether unilateral moyamoya disease, confirmed by typical angiographic evidence of moyamoya disease unilaterally and normal or equivocal findings contralaterally, is an early for...

how to cite this article: shiari r, tabatabaei nodushan smh, mohebbi mm, karimzadeh p, javadzadeh m. moyamoya syndrome associated with henoch-schönlein purpura. iran j child neurol. autumn 2016; 10(4):71-74. abstract some reports have shown the association between moyamoya syndrome and autoimmune diseases. herewith, we present a 3.5 yr old girl with henoch- schönleinpurpura (hsp) who was treate...

Moyamoya-like vasculopathy develops in association with various systemic diseases and conditions, which is termed moyamoya syndrome. Relatively common diseases and conditions are related to moyamoya syndrome, including neurofibromatosis type 1, Down syndrome, thyroid disease, and cranial irradiation. Moyamoya syndrome shares phenotypical characteristics with idiopathic moyamoya disease. However...

Moyamoya disease was first described in 1957 as hypoplasia of the bilateral internal carotid arteries, the characteristic appearance of the associated network of abnormally dilated collateral vessels on angiography was later likened to something hazy, like a puff of cigarette smoke, which, in Japanese, is moyamoya. This paper describes two cases of moyamoya presentations, including moyamoya dis...

Moyamoya disease is a progressive cerebrovascular occlusive disease that primarily affects children. The cause is unknown. We examined the production of prostanoids and the expression of cyclooxygenase-2 (COX-2) in cultured arterial smooth muscle cells (SMCs) derived from patients with moyamoya disease. Twelve moyamoya and 8 control cell strains were examined. The steady-state levels of prostan...

Moyamoya disease is characterized by a progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Although its etiology is still unknown, recent genome-wide and locus-specific association studies identified RNF213 as an important susceptibility gene of moyamoya disease among East Asian population. A polymorphism in c.14...