Evaluation of Cerebrovascular Reserve Using Basal/Acetazolamide Stress Brain Perfusion SPECT to Confirm Surgery in a Pediatric Moyamoya Disease.

We report the case of a 6-year-old girl with Moyamoya disease and Alagille syndrome (JAGGED1 mutation). She presented left hemiparesis and central left facial paralysis due to an ischemic stroke in the right middle cerebral artery territory. Basal/acetazolamide stress brain perfusion SPECT showed decreased cerebral perfusion and cerebrovascular reserve in all the carotid artery territory, which can be improved by indirect bypass surgery. Ten months after surgery, the child no longer had a stroke, general condition has improved significantly, as has her school performance. Control SPECT showed successful revascularization visualized by significant improvement in cerebral perfusion and cerebrovascular reserve.