{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,20]],"date-time":"2026-03-20T00:28:50Z","timestamp":1773966530154,"version":"3.50.1"},"reference-count":20,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["PED"],"published-print":{"date-parts":[[2015,7]]},"abstract":"<jats:sec>\n<jats:title>OBJECT<\/jats:title>\n<jats:p>Moyamoya can cause cerebral ischemia and stroke in Down syndrome (DS) patients. In this study, the authors defined a surgically treated population of patients with DS and moyamoya and compared their clinical presentation, response to surgical treatment, and long-term prognosis with those of the general population of patients with moyamoya but without DS.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>METHODS<\/jats:title>\n<jats:p>This study was a retrospective review of a consecutive operative series of moyamoya patients with DS treated at Boston Children\u2019s Hospital from 1985 through 2012.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>RESULTS<\/jats:title>\n<jats:p>Thirty-two patients, average age 9.7 years (range 1.8\u201329.3 years), underwent surgery for moyamoya in association with DS. The majority presented with ischemic symptoms (87% stroke, 42% transient ischemic attacks). Twenty-four patients (75%) had congenital heart disease. Nineteen patients (59%) had bilateral moyamoya on presentation, and 13 presented with unilateral disease, of which 2 progressed to surgery on the opposite side at a later date. Patients were followed for a median of 7.5 years (1\u201320.2 years) after surgery, with no patients lost to follow-up. Follow-up arteriography demonstrated Matsushima Grade A collaterals in 29 of 39 (74%) hemispheres, Grade B in 5 (13%), and Grade C in 5 (13%).<\/jats:p>\n<jats:p>Complications included postoperative strokes in 2 patients, which occurred within 48 hours of surgery in both; one of these patients had arm weakness and the other confusion (both had recovered completely at follow-up). Seizures occurred in 5 patients perioperatively, including one who had a new seizure disorder related to hypocalcemia.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>CONCLUSIONS<\/jats:title>\n<jats:p>Moyamoya disease is a cause of stroke in patients with DS. Both the incidence of preoperative stroke (87% vs 67%) and the average age at diagnosis for children under age 21 (8.4 vs 6.5 years) were greater in patients with DS and moyamoya than in the general moyamoya surgical population, suggesting a possible delay in reaching a correct diagnosis of the cause of cerebral ischemia in the DS patient population. Pial synangiosis provided long-term protection from stroke in all patients treated.<\/jats:p><\/jats:sec>","DOI":"10.3171\/2014.12.peds14563","type":"journal-article","created":{"date-parts":[[2015,4,3]],"date-time":"2015-04-03T12:57:21Z","timestamp":1428065841000},"page":"58-63","source":"Crossref","is-referenced-by-count":38,"title":["Down syndrome and moyamoya: clinical presentation and surgical management"],"prefix":"10.3171","volume":"16","author":[{"given":"Alfred P.","family":"See","sequence":"first","affiliation":[{"name":"Departments of Neurological Surgery and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexander E.","family":"Ropper","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel L.","family":"Underberg","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard L.","family":"Robertson","sequence":"additional","affiliation":[{"name":"Radiology, Boston Children\u2019s Hospital and Harvard Medical School, Boston, Massachusetts"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R. 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