A boy with infantile-onset fibromuscular dysplasia showing recurrent cerebrovascular attacks
Nozaki, Y.; Mizuguchi, M.; Momoi, M.Y.
No to Hattatsu 35(3): 253-258
2003
ISSN/ISBN: 0029-0831 PMID: 12755058 Document Number: 562246
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.