Postintravenous immunoglobulin stroke in a toddler with Down syndrome: a diagnostic challenge

Teck-Hock Toh1,2,3, Everlyn Coxin Siew4,3, Chae-Hee Chieng4,3

  • 1Department of Paediatrics, Sibu Hospital, Ministry of Health Malaysia, Sibu, Sarawak, Malaysia tohth@moh.gov.my.

BMJ Case Reports
|May 21, 2020
PubMed

Insights

Children with Down syndrome receiving intravenous immunoglobulin (IV Ig) may develop stroke. This case highlights the potential link between IV Ig and stroke in Down syndrome, suggesting pre-treatment cerebral vessel imaging.

Area of Science:

  • Neurology
  • Pediatrics
  • Immunology

Background:

  • Children with Down syndrome (DS) have an increased risk of stroke.
  • Intravenous immunoglobulin (IV Ig) therapy is associated with cerebrovascular events.
  • This report focuses on a pediatric case linking IV Ig and stroke in a child with DS.

Observation:

  • A 3-year-old boy with Down syndrome presented with severe pneumonia and received IV Ig.
  • Post-infusion, the child developed right hemiparesis, confirmed as acute left frontal and parietal infarcts via CT scan.
  • Cerebral vessel imaging revealed stenosis in the middle cerebral arteries and internal carotid arteries.

Findings:

  • Cerebral vascular imaging initially showed stenosis, with later findings suggestive of moyamoya disease.
  • The patient received anticoagulation therapy with enoxaparin and aspirin.
  • Partial recovery from hemiparesis was observed during follow-up.

Implications:

  • This case underscores the importance of considering moyamoya disease in children with Down syndrome and stroke.
  • Cerebral vessel imaging prior to IV Ig administration is recommended for high-risk pediatric patients.
  • Early detection and management may improve outcomes for stroke in this vulnerable population.