BACKGROUND AND PURPOSE BOLD MR imaging combined with a technique for precision control of end-tidal pCO(2) was used to produce quantitative maps of CVR in patients with Moyamoya disease. The technique was validated against measures of disease severity by using conventional angiography; it then was used to study the relationship between CVR, vascular steal, and disease severity. MATERIALS AND ...

Mutations in the ACTA2 gene lead to diffuse and diverse vascular diseases; the Arg179His mutation is associated with an early onset severe phenotype due to global smooth muscle dysfunction. Cerebrovascular disease associated with ACTA2 mutations has been likened to moyamoya disease, but appears to have distinctive features. This study involved the analysis of neuroimaging of 13 patients with he...

BACKGROUND AND PURPOSE Moyamoya syndrome is an uncommon chronic occlusive cerebrovascular disease in children. The origin of moyamoya syndrome remains undetermined. The role of the prothrombotic disorders contributing to its pathogenesis has not been completely elucidated. The purpose of this study was to determine the frequency of prothrombotic disorders in a pediatric population with moyamoya...

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...

It is well known that the electroencephalographic finding in patients with moyamoya disease demonstrates the characteristic "re-build up" phenomenon a few minutes after hyperventilation. To evaluate the usefulness of an electroencephalogram (EEG) in the postoperative management of children with moyamoya disease, we studied the presence or absence of improvement in the clinical, single photon em...

BACKGROUND AND PURPOSE We report the clinical features and longitudinal outcome of the largest cohort of patients with moyamoya disease described from a single institution in the western hemisphere. Moyamoya disease in Asia usually presents with ischemic stroke in children and intracranial hemorrhage in adults. METHODS Our study population included all patients with moyamoya disease evaluated...

RATIOINALE Relapsing polychondritis (RP) is a rare and heterogeneous disease complex of unknown origin which basically affects cartilaginous structures, 40% of which accompanied by rheumatic, hematologic, and endocrine disease. Among them, vasculitis is the most common accompanying type and usually presented with positive antineutrophilic cytoplasmic antibody (ANCA). The presence of ANCA could ...

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...

To cite: Gold JJ, Dory CE, Levy ML, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2013009812 DESCRIPTION A 17-year-old girl with a clinical diagnosis of neurofibromatosis type 1 (NF-1) presented for a routine follow-up with worsening headaches, leftsided hemiparesis and hyper-reflexia. Her medical history was significant for prematurity, speech delay and ...

Near-infrared spectroscopy (NIRS) was used to investigate the cerebral oxygenation of Moyamoya and healthy subjects. Continuous recordings of NIRS signals for 20 min (20 min signals) were obtained from 17 healthy subjects (age: 37.4 ± 11.3) and 17 Moyamoya subjects (age: 40.1 ± 11.2). Spectral analysis based on wavelet transformation identified five frequency intervals (I, 0.0095 Hz to 0.02 Hz;...