Introduction. Schimke immuno-osseous dysplasia (SIOD) is a rare autosomal recessive multisystem disorder associated with biallelic mutations of the SMAR-CAL1 gene. Vascular central nervous system complications in form Moyamoya syndrome (MMS) have been reported as comorbidity nearly half patients clinically presenting severe migraine-like headaches, transient ischemic attacks (TIA), and or hemor...

Here we report a case of moyamoya disease in which cortical veins reddened after superficial temporal artery (STA) to middle cerebral artery (MCA) anastomosis, following postoperative hyperperfusion. A 37-year-old man with moyamoya disease suffered cerebral infarction in his right hemisphere. Single photon emission computed tomography (SPECT) showed impaired cerebral blood flow (CBF) in both ce...

A 21-year-old woman presented with moyamoya disease manifesting as speech disturbance and right quadrant hemianopsia on October 22, 1994. Magnetic resonance (MR) angiography showed occlusion of the left internal carotid artery (ICA) with the normal right ICA. The diagnosis was "unilateral" moyamoya disease by conventional angiography. Follow-up MR angiography revealed further occlusive changes ...

Moyamoya disease is a chronic progressive vascular disease of brain characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation. We introduce here a patient with Moyamoya who was misdiagnosed. She was a 16-yr-old female from north east of Iran who complained left hemiparesis and was diagnosed Moyamoya disease by bra...

BACKGROUND AND PURPOSE In Moyamoya disease, the relationship between cerebral hemodynamics and angiographic findings has not been fully evaluated. The purpose of this study is to evaluate hemodynamics in Moyamoya disease with perfusion-weighted MR imaging (PWI) and cerebral angiography. METHODS Twenty patients with Moyamoya disease were the subjects. Mean transit time (MTT) derived from PWI w...

BACKGROUND AND PURPOSE Present criteria for the diagnosis of moyamoya disease emphasize the use of conventional cerebral angiography as an indispensable requirement. However, magnetic resonance angiography (MRA) is fast becoming a reliable diagnostic modality for occlusive cerebrovascular diseases. The purpose of this study is to assess the accuracy of MRA compared with conventional angiography...