An Adult Case of Noonan Syndrome Associated with Persistent Right Primitive Olfactory Artery and Vertebrobasilar Artery Hypoplasia

Yasushi Shibata


Noonan syndrome is genetic disease associated with cardiovascular anomalies. Mental retardation is frequently reported in patients with Noonan syndrome; however, reports of intracranial vascular anomalies are rare. Only several cases of Noonan syndrome-associated cerebrovascular disease have been reported. Most of these patients were children or young adults. We examined a nearly asymptomatic adult patient who was diagnosed with Noonan syndrome and found persistence of right primitive olfactory artery and vertebrobasilar artery hypoplasia. This is the first report of persistent primitive olfactory artery associated with Noonan syndrome. Noonan syndrome may be associated with various cerebrovascular diseases, even though there are no neurological symptoms; thus, the central nervous system and vascular system should be examined in patients with Noonan syndrome.


Noonan syndrome; Persistent primitive olfactory artery; Vertebrobasilar artery hypoplasia

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