Moyamoya disease: functional and neurocognitive outcomes in the pediatric and adult populations

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. (DOI: 10.3171/2011.3.FOCUS1150)