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Delayed Recognition and Stroke-Predominant Presentation in Down Syndrome-Associated Moyamoya Syndrome

Jonathan D Santoro1,2, Mackenzie Silverman1, Anthony C Wang3

  • 1Division of Neurology, Department of Pediatrics, Children's Hospital Los Angeles, CA (J.D.S., M.S., D.N., E.H., M.C.L., S.T.O., M.M.Y.).

Stroke
|July 24, 2026
PubMed

Insights

Children with Down syndrome (DS) and moyamoya syndrome (MMS) face a higher stroke risk and delayed diagnosis. Early detection strategies are crucial for this high-risk group.

Area of Science:

  • Neurology
  • Pediatrics
  • Vascular Medicine

Background:

  • Children with Down syndrome (DS) have an elevated risk for moyamoya syndrome (MMS) and ischemic stroke.
  • Early detection of MMS in DS patients is critical due to its surgical treatability, yet strategies remain undefined.

Purpose of the Study:

  • To compare the clinical characteristics and outcomes of children with DS-associated MMS (DS-MMS) versus MMS without DS.
  • To identify factors contributing to delayed diagnosis and assess neurological outcomes in DS-MMS.

Main Methods:

  • A multicenter retrospective cohort study comparing DS-MMS and MMS patients.
  • Outcomes included stroke presentation, diagnostic delays, angiographic findings, blood pressure, and 1-year neurological status.
  • Multivariable models adjusted for demographic and access-related factors.

Main Results:

  • Stroke at presentation was significantly more common in DS-MMS (71.4%) compared to MMS (20.7%).
  • DS-MMS patients experienced longer diagnostic delays and a higher frequency of posterior circulation involvement.
  • DS-MMS was associated with increased disability and spasticity at 1 year, alongside a progressive rise in systolic blood pressure percentiles pre-diagnosis.

Conclusions:

  • DS-MMS presents a high-risk cerebrovascular phenotype with delayed recognition and increased stroke likelihood.
  • Rising blood pressure percentiles may indicate early physiological changes in DS-MMS.
  • Findings support enhanced early detection and a lower threshold for vascular imaging in symptomatic DS patients.
Abstract

Keywords:
Down syndromeblood pressuremoyamoya diseaseodds ratiostroke

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