CADASIL: new cases and new questions.
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?
Rafalowska
J
J
Department of Neuropathology, Medical Research Centre, Polish Academy of Sciences, Pawinskiego 5, 02-106, Warsaw, Poland. ddziewul@amwaw.edu.pl
Fidziańska
A
A
Dziewulska
D
D
Podlecka
A
A
Szpak
G M
GM
Kwieciński
H
H
D000328:Adult; D001921:Brain / Q000098:blood supply* / Q000473:pathology* / Q000648:ultrastructure; D002536:Cerebral Arteries / Q000473:pathology* / Q000648:ultrastructure; D002560:Cerebrovascular Circulation; D015161:Dementia, Multi-Infarct / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D010375:Pedigree; D011044:Poland