Abstract
We present two patients with moyamoya syndrome and the unusual involvement of extracranial
vessels. The first case illustrates the rare association between moyamoya and primary
pulmonary hypertension. In the second patient, moyamoya was complicated by stenoses
of vertebral, renal, and mesenteric arteries. In both cases, a systemic intima-proliferative
disease, such as fibromuscular dysplasia (FMD), was suspected to be the cause of both
intracranial and extracranial arterial disease.
Key words
Moyamoya - fibromuscular dysplasia - pulmonary hypertension
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M. D., Ph.D. K. P. J. Braun
Department of Child Neurology
Wilhelmina Children's Hospital
University Medical Center Utrecht
P.O. Box 85090
3508 AB Utrecht
The Netherlands
Email: K.Braun@wkz.azu.nl
