CADASIL: new cases and new questions.

Abstract:

:We described the first two unrelated Polish families with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). In the morphological examination with light microscopy, two kinds of changes were observed: (1). panarteritis nodosa-like changes with eosinophilic fibrinoid necrosis of the vessel wall and perivascular inflammatory infiltrates and (2). basophilic granular material in the tunica media characteristic of CADASIL. At electron microscopy, we found deposits of granular osmophilic material (GOM) within the wall of arteries, veins and capillary vessels. Our findings imply two questions requiring further investigation: Why in the genetically determined vascular disorder are the features of systemic inflammatory vascular disease present? Why in capillary walls deprived of smooth muscle cells are deposits of GOM present?

journal_name

Acta Neuropathol

journal_title

Acta neuropathologica

authors

Rafalowska J,Fidziańska A,Dziewulska D,Podlecka A,Szpak GM,Kwieciński H

doi

10.1007/s00401-003-0764-4

subject

Has Abstract

pub_date

2003-12-01 00:00:00

pages

569-74

issue

6

eissn

0001-6322

issn

1432-0533

journal_volume

106

pub_type

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