Objective: To report unilateral leg freezing in a patient with moyamoya syndrome.

Background: Angiographic moyamoya is characterized by severe bilateral distal carotid stenosis with “puff-of-smoke” collateral vessels. The etiology can be idiopathic (moyamoya disease), or secondary to other causes including atherosclerosis (moyamoya syndrome).

Method: Case report.

Results: A 68-year-old Thai man with hypertension, diabetes and dyslipidemia initially developed acute left hemiparesis and facial weakness in 2005, and was diagnosed with ischemic stroke in the left middle cerebral artery territory. Detailed vascular studies were not performed at that time. He gradually recovered within a month, but had residual left hemiparesis (MRC grade 4). In 2015, after a mechanical fall, he developed stepwise progression of his gait. He denied problems with hand coordination, speech or swallowing. His mind remained clear. He did not seek medical attention until a year ago. Brain MRI revealed extensive subcortical white matter ischemic changes, more severe on the right. MRA demonstrated angiographic moyamoya, worse on the right. Given his age and vascular risk factors, this was likely due to intracranial atherosclerosis. Examination revealed lower body parkinsonism manifesting as freezing of gait (FOG), predominantly affecting the right leg, especially during gait initiation and turns, but also when walking straight. Base was wide. FOG was improved with visual and auditory cues. Despite left hemiparesis, there was no FOG of the left leg on the exam. There were no parkinsonian features in his arms and face. Leg movements were relatively good in seated position, compared to when walking, compatible with lower body parkinsonism. Given no response to levodopa 600 mg/day, pramipexole was added. A neurointervention for angiographic moyamoya were also recommended to prevent further vascular insults. However, this was unlikely to reverse his FOG from existing ischemic lesions. A video will be demonstrated.

Conclusion: Our case demonstrates unilateral right leg freezing as a unique phenotypic variant of lower body parkinsonism in the setting of moyamoya syndrome, likely secondary to intracranial atherosclerosis. This report expands movement disorders associated with moyamoya. Recognition of this phenomenology can be useful for searching an underlying vascular etiology, moyamoya syndrome in this case, which can have an impact on further management to prevent further vascular insults.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/unilateral-leg-freezing-in-moyamoya-syndrome/