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Moyamoya-like disease in Down's syndrome
1Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Md.
Pediatric Neurosurgery
|January 1, 1990
Summary
A child with Down syndrome experienced recurrent hemiparesis, a neurological condition. Angiography revealed moyamoya disease, characterized by carotid artery stenosis and collateral vessels.
Area of Science:
- Neurology
- Pediatrics
- Vascular Neurology
Background:
- Down syndrome is associated with an increased risk of cerebrovascular diseases.
- Moyamoya disease is a rare, progressive vascular disorder affecting brain arteries.
Observation:
- A 6-year-old girl with Down syndrome presented with recurrent episodes of hemiparesis.
- Neurological examination indicated focal neurological deficits.
Findings:
- Cerebral angiography demonstrated bilateral supraclinoid stenosis of the internal carotid arteries.
- Complexes of dilated collateral vessels were observed, characteristic of moyamoya disease.
Implications:
- This case highlights the importance of considering moyamoya disease in pediatric Down syndrome patients with unexplained neurological events.
- Early diagnosis and management of moyamoya disease are crucial to prevent stroke and improve outcomes in this population.