Cerebrovascular Accidents Resulting from Segmental Mediolytic Arteriopathy of the Cerebral Arteries in Young Adults

Abstract

Five cases of cerebrovascular accident or transient ischaemia in young adults resulting from segmental mediolytic arteriopathy are reported. The aetiology of segmental mediolytic arteriopathy is unknown but the disorder is characterized by focal, non-inflammatory mucoid degeneration of the media of muscular, non-arteriosclerotic arteries, frequently involving cerebral intracranial or extracranial vessels. It is similar to Erdheim—Gsell's medionecrosis of the aorta and may occur in any muscular artery. The consequences are mostly fatal in cerebral arteries. Histological analysis of each case is described. Clinicians should be aware of the possibility of intramural haemorrhage and dissecting aneurysm as a result of mucoid degeneration of the arterial media in young adults with cerebrovascular accident.

Keywords

cerebrovascular accident dissecting aneurysm segmental mediolytic arteriopathy mucoid degeneration of the arterial media

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References

Sandmann

Verdacht auf cerebrovaskuläre Durchblutungsstörung. Vortrag an der 25. Jahrestagung der Schweiz. Ges. f. Angiologie, Basel, 15–17, 10: 1992.

Bradac

, Kaernbach

, Bolk-Weischedel

Spontaneous dissecting aneurysms of cervical cerebral arteries. Neuroradiology 1981; 21: 149–54.

Anderson

RMcD

, Schechter

. A case of spontaneous dissecting aneurysm of the internal carotid artery. J Neurol Neurosurg Psychiatry 1959; 22: 195–201.

Boström

, Liliequist

Primary dissecting aneurysm of the extracranial part of the internal carotid and vertebral arteries. A report of three cases. Neurology 1967; 17: 179–86.

Brice

, Crompton

. Spontaneous dissecting aneurysm of the cervical internal carotid artery. BMJ 1964; ii(5412): 790–2.

Lloyd

, Bahnson

. Bilateral dissecting aneurysm of the internal carotid arteries. Am J Surg 1971; 122: 549–51.

Thapedi

, Ashenhurst

, Rozdilski

Spontaneous dissecting aneurysm of the internal carotid artery in the neck. Arch Neurol 1970; 23: 549–54.

Armas

, Donovan

. Segmental mediolytic arteritis involving hepatic arteries. Arch Pathol Lab Med 1992; 116: 531–4.

Slavin

, Gonzalez-Vitale

. Segmental mediolytic arteritis: a clinical pathologic study. Lab Invest 1976; 35: 23–9.

10.

Slavin

, Cafferty

, Cartwright

Segmental mediolytic arteritis: a clinicopathologic and ultrastructural study of two cases. Am J Surg Pathol 1989; 13: 558–68.

11.

Heritz

, Butany

, Johnston

, Sniderman

. Intra-abdominal hemorrhage as a result of segmental mediolytic arteritis of an omental artery. J Vasc Surg 1990; 12: 561–5.

12.

Gruenwald

Necrosis in the coronary artery of newborn patients. Am Heart J 1949; 38: 889–97.

13.

De Sa

. Coronary arterial and myocardial necrosis in stillbirths and infants. Arch Dis Child 1979; 54: 918–30.

14.

Lie

. Segmental mediolytic arteritis. Not an arteritis but a variant of arterial fibromuscular dysplasia. Arch Pathol Lab Med 1992; 116: 238–41.