Published Online
on July 27, 2006

A more recent version of this article appeared on September 1, 2006

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Submitted on May 31, 2006
Accepted on June 19, 2006

Arterioles of the Lenticular Nucleus in CADASIL

Qing Miao MSc; Timo Paloneva MD; Seppo Tuisku MD; Susanna Roine MD, PhD; Minna Poyhonen MD, PhD; Matti Viitanen MD, PhD; and Hannu Kalimo MD, PhD^*

From the Department of Pathology, University and University Hospital of Turku, Turku, Finland (Q.M., H.K.); the Department of Pathology, Keski-Pohjanmaa Central Hospital, Kokkola, Finland (T.P.); the Department of Neurology, Keski-Pohjanmaa Central Hospital, Kokkola, Finland (S.T.); the Department of Neurology, University and University Hospital of Turku, Turku, Finland (S.R.); the Department of Medical Genetics, University of Helsinki, Helsinki, Finland (M.P.); the Neurotec Department, Karolinska Institutet and Karolinska University Hospital Huddinge, Stockholm, Sweden (M.V.); the Department of Pathology, Uppsala Academic Hospital, Uppsala, Sweden (H.K.); the Department of Pathology, University and University Hospital of Helsinki, Helsinki, Finland (H.K.); the Department of Geriatric Medicine, University of Turku and Turku City Hospital, Turku, Finland (M.V.); the Department of Clinical Genetics, Helsinki University Hospital, Helsinki, Finland (M.P.).

^* To whom correspondence should be addressed. E-mail: hannu.kalimo{at}helsinki.fi.

Background and Purpose--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 -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.

Key words: CADASIL • cerebral arteries • fibrosis • lenticular nucleus • stenosis

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