Moyamoya syndrome in a pediatric NF-1 patient with underlying psychiatric diagnosis

Posted By Jonathan Mayl
Moyamoya syndrome in a pediatric NF-1 patient with underlying psychiatric diagnosis

Moyamoya syndrome is a rare cerebrovasculopathy of unknown etiology but is associated with multiple risk factors. Moyamoya was first discovered in Japan and is reported to have an increased prevalence in the Japanese population. The term moyamoya translates into puff of smoke and is named after the finding of the collateral cerebrovasculature that develops secondary to the occluded internal carotid artery at the entrance into the circle of Willis which characterizes this disease. Moyamoya should be included in the differential diagnosis in the pediatric population when a patient is presenting with stroke or stroke-like symptoms. Diagnosis can be made with cerebral angiogram or Magnetic resonance angiogram. Recent use of Magnetic resonance perfusion imaging, with a clinical correlation, has been shown to be useful in quantitatively assessing for the need for revascularization surgery. Considering our patients complex medical history of psychiatric illness and previously diagnosed neurofibromatosis, this modality proved instrumental in helping rule out moyamoya as the source of his worsening seizures and behavior. In our patient, it was determined that the relative perfusion for each side of the patient’s brain quantitatively lacked significant differences and he was therefore not a candidate for surgical revascularization.

Gender, Age

, 0

Leave A Comment

You need to be logged in to leave comments.

  • Jonathan Mayl 2016-09-29 01:00:41

    You are correct, Moyamoya is a know entity. However, this is an interesting case because of the co-morbid psychiatric condition, and NF-1 brought complexity to determining the proper therapeutic approach. Most importantly, this case is special to be publish because we used a combination of IVY sign on FLAIR, and MR-perfusion imaging modalities to determine if the worsening sx were due to moyamoya or the patients comorbid conditions. This information helped us determine the need, or lack thereof, for revascularization surgery in our patient.


  • Roland Talanow 2016-09-24 22:33:00

    Moyamoya is a known entity. What makes this case special to be published? Thank you.


  • Anonymous 2016-09-08 20:30:30

    Really interesting case! I look forward to seeing a full report.