Objective: To report on two cases of mixed movement disorder presentation of chorea and dystonia due to moyamoya syndrome for which one case was successfully treated with encephalo-duro-arterio-synangiosis (EDAS) procedure and the other with levodopa therapy.

Background: Two subjects ages 7- and 10-years-old presented with subacute-onset involuntary hyperkinetic movement disorders of chorea and dystonia that were asymmetric, affecting one hemibody more than the other.

Method: Both subjects underwent evaluation by neurological examination, serum laboratory investigation, MRI brain, and cerebral blood vessel imaging.

Results: Both subjects displayed subcortical T2 FLAIR white matter changes and terminal ICA stenosis, conferring a diagnosis of moyamoya disease. One subject was treated with levodopa therapy and had near cessation of dystonia and chorea. The other subject underwent EDAS procedure with resolution of symptoms within one month of surgery.

Conclusion: Movement disorders are not a common presentation of moyamoya syndrome, occurring in up to 10% of cases. Of the movement disorders that have been reported in moyomoya syndrome, dystonia has rarely been described. EDAS procedure is the mainstay of treatment. We report on the first case of levodopa-responsive chorea and dystonia in moyamoya disease and add to the literature two more cases of moyomoya-induced dystonia.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/two-cases-of-moyamoya-syndrome-associated-chorea-and-dystonia/