Stanford Moyamoya Publications

Moyamoya Disease and Pediatrics

Zhao, M. Y., Alexander, S., Lopez, C. A., Zhang, H., Morton, G., Armindo, R. D., Yeom, K. W., Tong, E., Soares, B. P., Lee, S., Moseley, M. and Steinberg, G. K. (2025). "Characterizing pre- and post-operative cerebral blood flow and transit time in pediatric moyamoya patients using multi-delay ASL and DSC MRI." J Cereb Blood Flow Metab: 271678X251358979.

Cerebral blood flow (CBF) and blood movement are key to brain health. This study examined 22 children with moyamoya disease using a method called multi-delay arterial spin labeling (ASL) alongside DSC MRI to assess changes before and after surgeries to improve blood flow. Results showed that after surgery, blood flow increased significantly (24% with ASL, 7.6% with DSC), and blood movement improved (12% with ASL, 15% with DSC). The close correlation between the two methods indicates that multi-delay ASL is effective for measuring blood flow in children.


Zhao, M. Y., Tong, E., Duarte Armindo, R., Fettahoglu, A., Choi, J., Bagley, J., Yeom, K. W., Moseley, M., Steinberg, G. K. and Zaharchuk, G. (2024). "Short- and Long-Term MRI Assessed Hemodynamic Changes in Pediatric Moyamoya Patients After Revascularization." J Magn Reson Imaging 59(4): 1349-1357.

This study examined 46 pediatric patients (ages 11 months to 18 years) with Moyamoya disease to assess changes in blood flow and cerebrovascular reserve (CVR) before and after bypass surgeries using a non-invasive MRI technique called arterial spin labeling (ASL). Imaging was done two weeks before, and one week and six months after surgery.

Results showed blood flow increased by 41% within a week and 51% after six months, CVR improved by 68%, and arterial transit time decreased by 6.6%. These findings indicate that bypass surgery can enhance blood flow and CVR in children with Moyamoya disease both in the short and long term.


Quon, J. L., Kim, L. H., MacEachern, S. J., Maleki, M., Steinberg, G. K., Madhugiri, V., Edwards, M. S. B., Grant, G. A., Yeom, K. W. and Forkert, N. D. (2020). "Early Diffusion Magnetic Resonance Imaging Changes in Normal-Appearing Brain in Pediatric Moyamoya Disease." Neurosurgery 86(4): 530-537.

This study examined brain diffusion changes in 29 children with moyamoya disease, who had normal pre-surgery MRIs, compared to 60 healthy peers. Results indicated significantly higher diffusion values in several brain areas of the children with moyamoya, despite no size differences. These findings suggest that even without visible stroke signs, children with moyamoya may experience altered brain water movement, indicating potential underlying blood flow issues.


Gupta, M., Choudhri, O. A., Feroze, A. H., Do, H. M., Grant, G. A. and Steinberg, G. K. (2016). "Management of moyamoya syndrome in patients with Noonan syndrome." J Clin Neurosci 28: 107-111.

This report highlights four cases of patients with Noonan syndrome who developed moyamoya syndrome, leading to headaches and strokes affecting one side of their bodies. Each patient underwent successful surgery to improve blood flow: a 10-year-old girl recovered well after a stroke, an adult with a childhood stroke improved but still had mini-strokes, a 7-year-old girl who didn't respond to medication had surgery, and a 24-year-old woman with worsening headaches had surgery to unblock an artery. These cases emphasize the importance of recognizing moyamoya syndrome in Noonan syndrome patients and the need for early surgical intervention to prevent further complications.


Pandey, P. and Steinberg, G. K. (2011). "Outcome of repeat revascularization surgery for moyamoya disease after an unsuccessful indirect revascularization. Clinical article." J Neurosurg 115(2): 328-336.

Revascularization surgery for moyamoya disease is effective in preventing strokes, but some patients may need repeat surgeries due to inadequate blood flow from initial indirect procedures. This study reviewed 16 patients (ages 5-48) who underwent repeat surgery after ongoing symptoms. Following the repeat procedures, 80% of surviving patients were free from new strokes or symptoms, and imaging showed improved blood flow in most cases. These results indicate that repeat revascularization can effectively assist patients with insufficient blood flow after initial treatments, with the choice of procedure based on the condition of the blood vessels.


Lee, M., Guzman, R., Bell-Stephens, T. and Steinberg, G. K. (2011). "Intraoperative blood flow analysis of direct revascularization procedures in patients with moyamoya disease." J Cereb Blood Flow Metab 31(1): 262-274.

Moyamoya disease narrows the main brain arteries, leading to strokes and bleeding, with surgery being the primary treatment. In a study of 292 patients who had 496 surgeries, we measured blood flow and vessel size. Average blood flow in a key artery was 4.4 mL/min before surgery and increased to 22.2 mL/min after bypass surgery. Children had lower blood flow than adults. Higher blood flow post-surgery was linked to better recovery, but very high flow rates increased the risk of stroke, bleeding, and temporary neurological issues. This data helps us understand the disease and the impact of surgery.


Pandey, P., Bell-Stephens, T. and Steinberg, G. K. (2010). "Patients with moyamoya disease presenting with movement disorder." J Neurosurg Pediatr 6(6): 559-566.

Moyamoya disease is a rare condition that narrows blood flow in the brain, usually causing mini-strokes or strokes, but it can also lead to movement disorders in children. This report highlights four pediatric patients who presented with movement issues, such as jerky movements and limb shaking, out of 446 treated patients. Brain scans showed reduced blood flow in the frontal brain area, and three had the disease on both sides. After undergoing surgery to improve blood flow, all four patients fully recovered, with most showing normal blood flow in follow-up scans. This underscores that movement disorders can be a rare symptom of moyamoya disease, and the surgery was effective in their treatment.


Guzman, R. and Steinberg, G. K. (2010). "Direct bypass techniques for the treatment of pediatric moyamoya disease." Neurosurg Clin N Am 21(3): 565-573.

Moyamoya is an increasingly recognized cause of stroke in children and adults. Identification of the disease early in its course with prompt institution of therapy is critical to providing the best outcome for patients. Revascularization surgery seems to be effective in preventing stroke in moyamoya, with direct techniques providing durable protection when performed at experienced centers.


Guzman, R., Lee, M., Achrol, A., Bell-Stephens, T., Kelly, M., Do, H. M., Marks, M. P. and Steinberg, G. K. (2009). "Clinical outcome after 450 revascularization procedures for moyamoya disease. Clinical article." J Neurosurg 111(5): 927-935.

Researchers at Stanford University Medical Center studied 329 patients with moyamoya disease (MMD) treated surgically between 1991 and 2008 to understand its characteristics and long-term outcomes in North America. The study included 233 adults (average age 39.5) and 96 children (average age 10.1), with most undergoing surgery to improve blood flow. Over an average follow-up of 4.9 years, the complication rate was low (3.5%) and the mortality rate was very low (0.7%), with a 5.5% chance of stroke or death within five years. Additionally, 91.8% of patients with temporary stroke-like symptoms were symptom-free after one year, and overall quality of life significantly improved. The results indicate that revascularization surgery for MMD is low-risk, effective in preventing future strokes, and enhances patients' quality of life.


Veeravagu, A., Guzman, R., Patil, C. G., Hou, L. C., Lee, M. and Steinberg, G. K. (2008). "Moyamoya disease in pediatric patients: outcomes of neurosurgical interventions." Neurosurg Focus 24(2): E16.

Surgical treatments for moyamoya disease (MMD) in children include direct, indirect, and combined methods, all of which have proven effective, but no study has shown one to be superior. This review analyzes various studies, highlighting results from different hospitals. Direct methods can quickly improve blood flow and reduce stroke risk but may not be suitable for all children due to their complexity. Indirect methods are also effective but may take longer to show results. Overall, it's unclear which technique is best, as most studies involved small patient groups and varied success measures. More extensive research is needed to understand the pros and cons of each technique for pediatric patients.


Karzmark, P., Zeifert, P. D., Tan, S., Dorfman, L. J., Bell-Stephens, T. E. and Steinberg, G. K. (2008). "Effect of moyamoya disease on neuropsychological functioning in adults." Neurosurgery 62(5): 1048-1051; discussion 1051-1042.

Moyamoya disease is a brain blood vessel disorder that leads to the closure of key vessels and the formation of abnormal blood vessels. This study evaluated 36 adults with confirmed moyamoya disease using neuropsychological tests before surgery. While overall cognitive performance was normal, significant difficulties were noted in executive functioning, with 31% of patients showing some cognitive impairment—four with moderate to severe issues. Memory and intelligence were largely unaffected, and some patients reported mild to moderate depression. These results suggest that moyamoya disease can impact cognition in adults, but the effects are generally less severe than in children, highlighting the need for further research on brain function.