Moyamoya Syndrome Manifesting with Choreiform Movements

Case Summary

An 11-year-old girl was referred to our department 20 days after the sudden onset of choreiform movements. She had no other complaints or underlying diseases. The movements compromised her right limbs and considerably impaired her education and daily activities. During physical examinations, we observed choreiform movements in her right limbs ([Video 1], available online-only), but muscle strength was preserved. No other abnormalities were observed. Magnetic resonance imaging revealed flow voids in her left basal ganglia and a left hemisphere hypoperfusion pattern ([Fig. 1]). Digital angiography revealed the “puff of smoke” sign resulting from abnormally dilated collateral vessels ([Fig. 2]). She was diagnosed as having moyamoya syndrome and underwent indirect revascularization.

Fig. 1 Brain magnetic resonance imaging (MRI) scan shows flow voids within the left basal ganglia (A, arrow). MRI perfusion indicates a hypoperfused left hemisphere.

Fig. 2 Digital angiogram shows a narrowed middle cerebral artery and a puff of smoke sign in the collateral vessels.

Video 1 The patient has choreiform movements in the right arm and leg. Online content including video sequences viewable at: www.thieme-connect.com/ejournals/https://doi.org/10.1055/s-0037-1607967.

Moyamoya syndrome is characterized by progressive narrowing of the intracranial carotid artery, leading to the compensatory development of collateral vessels.[1] Clinical symptoms are mostly related to ischemia. The ischemia and presence of abnormal vessels in the basal ganglia may lead to movement disorders.[2]

• References

• 1 Scott RM, Smith ER. Moyamoya disease and moyamoya syndrome. N Engl J Med 2009; 360 (12) 1226-1237
• 2 Ahn ES, Scott RM, Robertson Jr RL, Smith ER. Chorea in the clinical presentation of moyamoya disease: results of surgical revascularization and a proposed clinicopathological correlation. J Neurosurg Pediatr 2013; 11 (03) 313-319