Moyamoya disease (MMD) is an arteriopathy of the intracranial circulation predominantly affecting the branches of the internal carotid arteries. Heterogeneity in presentation, progression and response to therapy has prompted intense study to improve the diagnosis and prognosis of this disease. Recent progress in the development of moyamoya-related biomarkers has stimulated marked interest in th...

CONTEXT Neurofibromatosis type 1 (NF-1) is the most prevalent autosomal dominant genetic disorder among humans. Moyamoya disease is a cerebral vasculopathy that is only rarely observed in association with NF-1, particularly in the pediatric age range. The present study reports an occurrence of this association in an infant. CASE REPORT An eight-month-old female presented convulsive seizures w...

BACKGROUND Arteriovenous malformations (AVMs) with vascular abnormalities, including aneurysms, have been reported frequently. However, the coexistence of AVM and unilateral moyamoya disease is rare. We report herein an AVM patient who presented with acute ischemic stroke with unilateral moyamoya disease and occlusion of the feeding artery. CASE REPORT A-41-year old man was admitted with sudd...

PURPOSE To describe the angiographic changes accompanying the surgical treatment of moyamoya disease by pial synangiosis and to compare these changes with patient outcome. METHODS The preoperative and postoperative cerebral angiograms, MR images, and clinical records of 13 children treated with pial synangiosis for moyamoya disease were reviewed. RESULTS After synangiosis, 10 patients had s...

Moyamoya disease is a disease of the cerebral vessels, which is characterized by narrowing of the intracranial vessels as they emerge from the base of the skull, and hypertrophy of fine collateral vessels in response to this vessel narrowing. Moyamoya means the puff of smoke in Japanese, and refers to the appearance of the abnormal fine collateral vessels appearing on cerebral angiography in pa...

See related article, p 1245. Moyamoya disease is a unique cerebrovascular disease characterized by progressive occlusion of the bilateral internal carotid arteries at their terminal portions and unusual secondarily formed vascular networks (moyamoya vessels) that act as collateral pathways. Unlike pediatric patients, who usually present with transient ischemic attacks (TIAs) or cerebral infarct...

Moyamoya disease is infrequently associated with intracranial aneurysms arising from the circle of Willis vessels or from "peripheral" branches of choroidal and meningeal vessels. We present a rare case of a moyamoya-related aneurysm arising along the dural junction of multiple meningeal branches from the external carotid artery causing intracerebral hemorrhage. Endovascular coil embolization o...

We conducted a retrospective study to determine whether the presence of moyamoya collaterals influenced the risk of recurrence of cerebrovascular events (CVEs: stroke or transient ischemic attack) in patients with sickle cell disease placed on chronic transfusions after a stroke. Forty-three patients with homozygous sickle cell anemia (HbSS) and 1 with HbSO(Arab) (16 females, 28 males) who had ...

One hundred cases of Moyamoya disease were encountered between 1961 and 1980. This report describes the clinical characteristics and emphasizes the angiographic findings and clinical correlation in this disease. Reasons for the differences in clinical and radiological presentation in children versus adults are proposed and a possible pathophysiological mechanism is outlined. Treatment with peri...

Moyamoya disease occurring in Chinese has been inadequately described. Here we report 13 cases of this disease identified by review of 3,200 cerebral angiograms performed between August 1979 and March 1986. Nine were males and four were females; there were 12 adults (aged 34-51 years) and one child (aged 11 years). All had hemorrhagic strokes with one exception, a patient with an occipital infa...