Objective: To report the rapid reduction of disabling chorea in two cases of pediatric Moyamoya disease with pulse steroid therapy.

Background: The common clinical presentation of paediatric Moyamoya disease is transient ischemic attacks, seizures, and migraine like headaches. Movement disorders occur in 3-6% of pediatric Moyamoya disease and chorea is the commonest of all. Most often, Chorea is highly disabling and interferes with the activities of daily living. We present to you two cases of chorea, secondary to Moyamoya disease which were highly steroid responsive.

Method: case report

Results: Case 1: A 8-year-old female with normal birth history & developmental milestones came with history of generalized seizures, cognitive decline and generalized chorea (left>right) since 2 years & interfering with her daily activities since 1 year. The workup for childhood onset chorea revealed MRI with MRA showing Suzuki grade 3-4 Moyamoya disease. She was treated with IV pulse methyl prednisolone for 3 days and UFMG Sydenham Chorea Rating Scale decreased from 48/84 to 32/84 immediately post 3 days of steroids Case 2: A 9-year-old female presented with recurrent left hemiparesis, seizure disorder and generalized choreiform movements since 2 years. MRI with MRA and Digital subtraction angiography confirmed Moyamoya disease as in [figure 1]. She was also treated with 3 days of IV pulse methylprednisolone, after which her UFMG Sydenham’s Chorea rating scale decreased from 24/84 to 3/84 and was able to carry out her daily activities.

Conclusion: Chorea occurs in Moyamoya disease probably due to the ischemic changes affecting the excitatory-inhibitory circuits connecting the basal ganglia and the cerebral cortex. Chorea often subsides with revascularization surgeries according to the previous studies. So our two cases highlight the need of steroids as a bridging therapy before surgery in view of the high reluctance or longer wait times to surgery. There were only 2 separate case reports of response to steroids in chorea with Moyamoya disease in the literature. Though the etiology of Moyamoya disease is unknown, steroids probably decrease the chorea by affecting the neurotransmitters in the basal ganglia or some restoration of perfusion after steroid therapy. Further studies are required to elucidate the mechanism behind the response to steroids.

References: 1. Pavlakis, Steven G., et al. “Steroid-Responsive Chorea in Moyamoya Disease.” Movement Disorders, vol. 6, no. 4, 1991, pp. 347–349 2. Ahn, Edward S., et al. “Chorea in the Clinical Presentation of Moyamoya Disease: Results of Surgical Revascularization and a Proposed Clinicopathological Correlation.” Journal of Neurosurgery: Pediatrics, 2013, pp. 313–319 3. vittal P et al. “Steroids- An initial symptomatic treatment for Moyamoya Induced chorea.” Movement disorders 2013 abstract supplement.

« Back to 2019 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/steroid-responsive-chorea-in-moyamoya-disease-illustrative-cases/