Fibromuscular dysplasia and multiple dissecting aneurysms of intracranial arteries. A further cause of Moyamoya syndrome. 1976

P Pilz, and H J Hartjes

A 16-year-old boy, who had sudden left-sided hemiplegia, died two weeks following onset of symptoms. A right carotid angiogram showed stenosis at the termination of the internal carotid artery. The middle cerebral artery had a beaded appearance and some of its branches were occluded. A basal "moyamoya" syndrome and transdural anastomoses were present. At autopsy, multiple intracranial dissecting aneurysms were found. Arteries of the body displayed fibromuscular dysplasia (FMD). The relevance of dysplastic changes of intracranial arteries and the relationship to moyamoya syndrome are discussed.

UI MeSH Term Description Entries
D008297 Male Males
D011859 Radiography Examination of any part of the body for diagnostic purposes by means of X-RAYS or GAMMA RAYS, recording the image on a sensitized surface (such as photographic film). Radiology, Diagnostic X-Ray,Roentgenography,X-Ray, Diagnostic,Diagnostic X-Ray,Diagnostic X-Ray Radiology,X-Ray Radiology, Diagnostic,Diagnostic X Ray,Diagnostic X Ray Radiology,Diagnostic X-Rays,Radiology, Diagnostic X Ray,X Ray Radiology, Diagnostic,X Ray, Diagnostic,X-Rays, Diagnostic
D002343 Carotid Artery, Internal Branch of the common carotid artery which supplies the anterior part of the brain, the eye and its appendages, the forehead and nose. Arteries, Internal Carotid,Artery, Internal Carotid,Carotid Arteries, Internal,Internal Carotid Arteries,Internal Carotid Artery
D002532 Intracranial Aneurysm Abnormal outpouching in the wall of intracranial blood vessels. Most common are the saccular (berry) aneurysms located at branch points in CIRCLE OF WILLIS at the base of the brain. Vessel rupture results in SUBARACHNOID HEMORRHAGE or INTRACRANIAL HEMORRHAGES. Giant aneurysms (>2.5 cm in diameter) may compress adjacent structures, including the OCULOMOTOR NERVE. (From Adams et al., Principles of Neurology, 6th ed, p841) Aneurysm, Cerebral,Aneurysm, Intracranial,Basilar Artery Aneurysm,Berry Aneurysm,Brain Aneurysm,Cerebral Aneurysm,Giant Intracranial Aneurysm,Mycotic Aneurysm, Intracranial,Aneurysm, Anterior Cerebral Artery,Aneurysm, Anterior Communicating Artery,Aneurysm, Basilar Artery,Aneurysm, Middle Cerebral Artery,Aneurysm, Posterior Cerebral Artery,Aneurysm, Posterior Communicating Artery,Anterior Cerebral Artery Aneurysm,Anterior Communicating Artery Aneurysm,Middle Cerebral Artery Aneurysm,Posterior Cerebral Artery Aneurysm,Posterior Communicating Artery Aneurysm,Aneurysm, Berry,Aneurysm, Brain,Aneurysm, Giant Intracranial,Aneurysm, Intracranial Mycotic,Aneurysms, Basilar Artery,Aneurysms, Berry,Aneurysms, Brain,Aneurysms, Cerebral,Aneurysms, Giant Intracranial,Aneurysms, Intracranial,Aneurysms, Intracranial Mycotic,Artery Aneurysm, Basilar,Artery Aneurysms, Basilar,Basilar Artery Aneurysms,Berry Aneurysms,Brain Aneurysms,Cerebral Aneurysms,Giant Intracranial Aneurysms,Intracranial Aneurysm, Giant,Intracranial Aneurysms,Intracranial Aneurysms, Giant,Intracranial Mycotic Aneurysm,Intracranial Mycotic Aneurysms,Mycotic Aneurysms, Intracranial
D002536 Cerebral Arteries The arterial blood vessels supplying the CEREBRUM. Arteries, Cerebral,Artery, Cerebral,Cerebral Artery
D002561 Cerebrovascular Disorders A spectrum of pathological conditions of impaired blood flow in the brain. They can involve vessels (ARTERIES or VEINS) in the CEREBRUM, the CEREBELLUM, and the BRAIN STEM. Major categories include INTRACRANIAL ARTERIOVENOUS MALFORMATIONS; BRAIN ISCHEMIA; CEREBRAL HEMORRHAGE; and others. Brain Vascular Disorders,Intracranial Vascular Disorders,Vascular Diseases, Intracranial,Cerebrovascular Diseases,Cerebrovascular Insufficiency,Cerebrovascular Occlusion,Brain Vascular Disorder,Cerebrovascular Disease,Cerebrovascular Disorder,Cerebrovascular Insufficiencies,Cerebrovascular Occlusions,Disease, Cerebrovascular,Diseases, Cerebrovascular,Insufficiencies, Cerebrovascular,Insufficiency, Cerebrovascular,Intracranial Vascular Disease,Intracranial Vascular Diseases,Intracranial Vascular Disorder,Occlusion, Cerebrovascular,Occlusions, Cerebrovascular,Vascular Disease, Intracranial,Vascular Disorder, Brain,Vascular Disorder, Intracranial,Vascular Disorders, Brain,Vascular Disorders, Intracranial
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D001158 Arteries The vessels carrying blood away from the heart. Artery
D013577 Syndrome A characteristic symptom complex. Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes

Related Publications

P Pilz, and H J Hartjes
August 2010, International journal of stroke : official journal of the International Stroke Society,
P Pilz, and H J Hartjes
January 1960, Neurology,
P Pilz, and H J Hartjes
January 2020, Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology,
P Pilz, and H J Hartjes
February 1970, The Journal of pathology,
Copied contents to your clipboard!