Arterioles of the lenticular nucleus in CADASIL.
OBJECTIVE
In cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) the arteriopathy leads to recurrent infarcts in cerebral white matter (WM) and deep gray matter (GM), whereas cortex is spared. To assess the pathogenesis of deep GM infarcts, we analyzed structural changes in arterioles of the lenticular nucleus (LN) in 6 CADASIL patients.


METHODS
Five elderly and one 32-year-old deceased CADASIL patients were studied. Seven elderly and 4 young deceased persons without cerebrovascular diseases served as controls. In addition to immunohistochemical analysis the external and luminal diameters of arterioles in the LN, cerebral cortex and WM were measured. The thickness of arteriolar wall and sclerotic index were calculated.


RESULTS
In CADASIL patients, LN arterioles were immunoreactive for the extracellular domain of Notch3 and collagen I, whereas alpha-smooth muscle actin staining was irregular or negative. No major leakage of plasma fibrinogen or fibronectin was observed. Although in patients the walls of LN arterioles were significantly thicker than in controls, definite stenosis was not observed. Arteriolar lumina in the LN were not only significantly larger than in the WM, where most lacunar infarcts in CADASIL occur, but also larger than in cortical GM, where infarcts virtually never exist.


CONCLUSIONS
Fibrotic thickening of the arteriolar walls without consequent stenosis occurs in the LN of CADASIL patients. The pathogenesis of lacunar infarcts in the WM and LN seem to be different, stenosis in the former and probably hemodynamic disturbances in the latter.
Miao
Qing
Q
Department of Pathology, University and University Hospital of Turku, Turku, Finland.
Paloneva
Timo
T
Tuisku
Seppo
S
Roine
Susanna
S
Poyhonen
Minna
M
Viitanen
Matti
M
Kalimo
Hannu
H
D000328:Adult; D000368:Aged; D001160:Arterioles / Q000378:metabolism / Q000473:pathology; D046589:CADASIL / Q000378:metabolism / Q000473:pathology*; D002102:Cadaver; D016022:Case-Control Studies; D003342:Corpus Striatum / Q000098:blood supply*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D010487:Periaqueductal Gray / Q000473:pathology; D012598:Sclerosis