Endovascular management of extracranial arterial dissection continues to evolve. Current experience shows that this treatment option is safe and technically feasible. Prospective randomized trials compared with medical management are needed to further elucidate the role of stenting.

Adenosine appears to allow safe flow arrest during intracranial aneurysm surgery. This can enhance the feasibility and safety of clipping in select circumstances.

Object Moyamoya disease (MMD) is a rare cerebrovascular disorder involving stenosis of the major vessels of the circle of Willis and proximal portions of its principal branches. Despite concerted investigation, the pathophysiology of the disorder has not been fully elucidated. Currently, the major proteins believed to play an active role in the pathogenesis include vascular endothelial growth factor (VEGF), basic fibroblast growth factor (bFGF), hepatocyte growth factor (HGF), transforming growth factor–β1 (TGFβ1), and granulocyte colony-stimulating factor (G-CSF). In terms of the genetics, recent literature suggests a low penetrance autosomal dominant or polygenic mode of transmission involving chromosomes 3, 6, 8, 12, and 17 for familial MMD. This review summarizes the current knowledge on the histopathology, pathophysiology and genetics of MMD. Methods A PubMed/Medline systematic study of the literature was performed, from which 45 articles regarding MMD pathophysiology were identified and analyzed. Conclusions Moyamoya disease is characterized by the intimal thickening and media attenuation of the proximal vessels of the circle of Willis as well as the development of an aberrant distal vascular network. The primary proteins that are currently implicated in the pathophysiology of MMD include VEGF, bFGF, HGF, TGFβ1, and G-CSF. Furthermore, the current literature on familial MMD has pointed to a low penetrance autosomal dominant or polygenic mode of transmittance at loci on chromosomes 3, 6, 8, 12, and 17.

Object Moyamoya disease is an occlusive cerebrovascular disorder commonly resulting in neurocognitive impairment. The cognitive outcome parameters commonly affected are intelligence, memory, executive function, and quality of life. In this paper, the authors review the existing literature on cognitive and clinical outcomes in adult and pediatric moyamoya populations separately. Methods A systematic review of the cognitive and clinical outcome literature was performed using the PubMed/MEDLINE database. Outcomes data were contrasted between adult and pediatric populations. Results Intelligence is the main cognitive outcome parameter affected in pediatric patients with moyamoya disease, whereas adults most commonly suffer from executive function impairment. Memory has not been studied sufficiently in pediatric patients, and its dysfunction in the adult population remains controversial. Quality of life has not been studied appropriately in either population. Surgical revascularization is the only beneficial treatment option, and a combination of direct and indirect bypass techniques has shown benefit, but the impact on the above-mentioned parameters has not been sufficiently elucidated. Conclusions Moyamoya disease affects the cognition and daily function in pediatric patients to a greater extent than in adult patients. Due to the rarity of the disease, there is a distinct lack of high-level evidence regarding cognitive and clinical outcomes.

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