Chorea in the clinical presentation of moyamoya disease: Results of surgical revascularization and a proposed clinicopathological correlation

Edward S. Ahn, R. Michael Scott, Richard L. Robertson, Edward R. Smith

Research output: Contribution to journalArticlepeer-review


Object. Chorea is a movement disorder characterized by brief, irregular, involuntary contractions that appear to flow from 1 muscle to another. There are a limited number of reports in the literature that have linked moyamoya disease and chorea. The authors describe their experience in treating moyamoya disease in patients in whom chorea developed as part of the clinical presentation. Methods. The authors conducted a retrospective review of a consecutive series of 316 children who underwent pial synangiosis revascularization for moyamoya disease at the Boston Children's Hospital. Results. Of 316 surgically treated patients with moyamoya disease, 10 (3.2%; 6 boys and 4 girls) had chorea as a part of their presentation. The average age at surgical treatment was 9.9 years (range 3.8-17.9 years). All patients had evidence of hypertrophied lenticulostriate collateral vessels through the basal ganglia on preoperative angiography and/or MRI on affected sides. Two patients had cystic lesions in the basal ganglia. Nine patients underwent bilateral craniotomies for pial synangiosis, and 1 patient underwent a single craniotomy for unilateral disease. Follow-up was available in 9 patients (average 50.1 months). The mean duration of chorea was 1.36 years (range 2 days to 4 years), with resolution of symptoms in all patients. One patient developed chorea 3 years after surgical treatment, 4 patients had transient chorea that resolved prior to surgery, and 5 patients experienced resolution of the chorea after surgery (average 13 months). Conclusions. The authors describe children with moyamoya disease and chorea as part of their clinical presentation. The data suggest that involvement of the basal ganglia by the hypertrophied collateral vessels contributes to the development of chorea, which can wax or wane depending on disease stage or involution of the vessels after revascularization surgery. In most patients, however, the chorea improves or disappears about 1 year after presentation.

Original languageEnglish (US)
Pages (from-to)313-319
Number of pages7
JournalJournal of Neurosurgery: Pediatrics
Issue number3
StatePublished - Mar 2013


  • Chorea
  • Dyskinesia
  • Movement disorder
  • Moyamoya disease
  • Pial synangiosis
  • Surgical revascularization vascular disorders

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology


Dive into the research topics of 'Chorea in the clinical presentation of moyamoya disease: Results of surgical revascularization and a proposed clinicopathological correlation'. Together they form a unique fingerprint.

Cite this