Summary
Degenerative processes of the tunica media are reported with increasing frequency among the causes of a dissecting aneurysm in the wall of large arteries. The underlying process is supposed to be a necrosis or degeneration of elastic tissue and smooth muscle cells, occurring either in idiopathic necrosis of the media (Gsell-Erdheim) or in systemic disorders as Marfan's syndrome, pseudoxanthoma elasticum, Ehlers-Danlos-syndrome and homocystinuria. Neurological symptoms in these conditions are attributed to disturbances of general circulation but not to involvement of cerebral arteries.
The findings in 3 young patients are reported (1 Marfan's syndrome, 2 patients with medianecrosis of large arteries) exhibiting the characteristic histological changes in the media of the cerebral arteries, too. Possible connections between such degenerative diseases of the tunica media and the origin of fusiform aneurysms or the “megadolicho-basilaris syndrome” of cerebral arteries are suggested.
Literatur
Austin, M. G., Schaefer, R. F.: Marfan's Syndrome with unusual blood vessel manifestations. Arch. Path.64, 205–209 (1957)
Boquist, L., Berg, P.: Multiple dissecting aneurysm, in peripheral arteries. J. Path. Bact.100, 145–148 (1970)
Carey, M. C., Donovan, D. E., Fitzgerald, O., McAuley, F. D.: Homocystinuria. Amer. J. Med.45, 7–25 (1968)
Cornette, M., Focan, C., Schyus, P., Franck G.: Syndrome de Marfan, stries angiooides de la rétine et comitialité. Acta neurol. belg.71, 445–452 (1971)
Eschbach, O., Zülch, K. J.: Radiologie1, 415–418 (1969)
Foord, A. G., Lewis, R. D.: Primary dissecting aneurysm of peripheral and pulmonary arteries: dissecting hemorrhage of media. Arch. Path.68, 553–557 (1959)
Goodman, R. M., Baba, N., Wooley, Ch. F.: Observations of the heart in a case of combined Ehlers-Danlos and Marfan Syndromes. Amer. J. Cardiol.24, 734–742 (1969)
Gore, I.: Dissecting aneurysms of the aorta in persons under forty years of age. Arch. Path.55, 1 (1953)
Guerrero, E.: Primary dissecting aneurysm of the hepatic artery. Arch. Path.89, 569–573 (1970)
Hirst, A. E., Johns, V. J., Kime, S. W.: Dissecting aneurysm of the aorta: A review of 505 cases. Medicine (Baltimore)37, 217–279 (1958)
Hueck, W.: Morphologische Pathologie., Leipzig: Thieme 1955
Ley, A.: Compression of the optic nerve by a fusiform aneurysm, of the carotid artery. J. Neurol. Psychiat.13, 75–86 (1950)
Peiper, H.-J.: Aneurysmen der thorakalen Aorta. Thorax-Chirurgie-Vaskuläre Chirurgie, Bd. 16, S. 414–421. Stuttgart: Thieme 1968
Rahn, J., Schieche, M., Radestock, G.: Mediomalacia vasculativa aortae: Ursachen. Folgen und Formen. Dtsch. Gesundh.23, 2240–2246 (1968)
Rubinstein, M. K., Cohen, N. H.: Ehler-Danlos-syndrome associates with multiple intracranial aneurysms. Neurology (Minneap.)14, 125 (1964)
Schmidt, M. B.: Über die Schlängelung der Arteria temporalis. Zbl. allg. Path. path. Anat.30, 49–56 (1919)
Shiro, I., Robin, M., Bannerman, G. J., Brennan, C. J.: Ehlers-Danlos-syndrome with multiple arterial lesions. Amer. J. Med.47, 967–977 (1969)
Shilkin, K. B., Low, L. P., Chen, B. T. M: Dissecting aneurysm of the pulmonary artery. J. Path. Bact.98, 25–29 (1969)
Staemmler, M.: In: Kaufmann-Staemmler: Spez. Path., Bd. I/1. Berlin: W. de Gruyter 1955
Volberg, G.: Untersuchungen zur Frage der Kombination von Hirngefäßaneurysmen mit anderen Mißbildungen. Diss., Düsseldorf 1969
Waberzinek, G., Urbánek, K., Klaus, E.: Beitrag zum klinischen Bild der sog. Megadolichobasilaris. Nervenarzt42, 208–211 (1971)
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Gerhard, L., Schmitz-Bauer, G. Hirnbasisarterienveränderungen bei Marfan-Syndrom und idiopathischer Media-Nekrose. Acta Neuropathol 26, 179–184 (1973). https://doi.org/10.1007/BF00697753
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DOI: https://doi.org/10.1007/BF00697753