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.