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Updated: Sep 26, 2026

Longitudinal In Vivo Imaging of the Cerebrovasculature: Relevance to CNS Diseases
Published on: December 6, 2016
[A boy with infantile-onset fibromuscular dysplasia showing recurrent cerebrovascular attacks]
Yasuyuki Nozaki1, Masashi Mizuguchi, Mariko Y Momoi
1Department of Pediatrics, Jichi Medical School, Minamikawachi-gun, Tochigi. nozakiy@jichi.ac.jp
Insights
This case study details a boy with early-onset fibromuscular dysplasia (FMD), a rare arterial disease. The condition caused severe cerebrovascular and systemic artery issues, highlighting FMD
Area of Science:
- Pediatric Cardiology
- Vascular Biology
- Neurology
Background:
- Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory vascular disease.
- Infantile-onset FMD is exceedingly rare, with limited case reports.
- Understanding early-onset FMD is crucial for pediatric vascular health.
Observation:
- A male infant presented with cerebrovascular events at 8 months.
- Symptoms included facial palsy, hemiparesis, and intestinal ischemia.
- Systemic artery involvement caused limb underdevelopment and renovascular hypertension.
Findings:
- Cerebral angiography revealed bilateral internal carotid and right vertebral artery stenosis.
- Systemic arteriography showed stenosis in brachial and femoral arteries.
- The FMD presented with early onset, multifocal arterial involvement, and severe symptoms.
Implications:
- This case underscores the potential severity of infantile-onset FMD.
- Early diagnosis and management are critical for preventing complications.
- Further research is needed to elucidate the pathogenesis of early-onset FMD.
Abstract:
We report here a boy with infantile-onset fibromuscular dysplasia (FMD). At 8 months of age, he had the first cerebrovascular attack presenting with left facial palsy, followed by recurrent attacks of alternating hemiparesis. Involvement of systemic arteries was manifested by variable somatic symptoms: retarded growth of the right arm due to stenosis of the brachial artery, constipation and recurrent abdominal pain due to intestinal ischemia, and renovascular hypertension. Cerebral angiography disclosed stenosis of the bilateral internal carotid arteries and right vertebral artery, with development of moyamoya disease like collateral vessels. Systemic arteriography revealed stenosis of the right brachial and left femoral arteries, which was either tubular or mimicking a string of beads. This case was characterized by the early onset and involvement of many cerebral and systemic arteries resulting in severe and variable symptoms.
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