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Acta Neuropathologica

, Volume 26, Issue 2, pp 179–184 | Cite as

Hirnbasisarterienveränderungen bei Marfan-Syndrom und idiopathischer Media-Nekrose

  • Lieselotte Gerhard
  • Gabriele Schmitz-Bauer
Kurze Originalmitteilungen

Morphology of cerebral arteries in Marfan's syndrome and medianecrosis idiopathica

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.

Key words

Cerebral Arteries Fusiform Aneurysm Medianecrosis idiopathica Marfan's Syndrome Megadolichobasilaris 

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Copyright information

© Springer-Verlag 1973

Authors and Affiliations

  • Lieselotte Gerhard
    • 1
  • Gabriele Schmitz-Bauer
    • 1
  1. 1.Pathologisches Institut der Universität DüsseldorfDüsseldorfBundesrepublik Deutschland

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