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Lookup NU author(s): Dr Lucinda Craggs,
Dr Vincent Deramecourt,
Professor Raj Kalaria
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We quantified vascular changes in the frontal lobe and basal ganglia of four inherited small vessel diseases (SVDs) including cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), pontine autosomal dominant microangiopathy and leukoecephalopathy (PADMAL), hereditary multi-infarct dementia of Swedish type (Swedish hMID) and hereditary endotheliopathy with retinopathy, nephropathy, and stroke (HERNS). Vascular pathology was most severe in CADASIL, and varied with marginally greater severity in the basal ganglia compared to the frontal lobe. The overall sclerotic index values in frontal lobe were in the order CADASIL > HERNS > PADMAL > Swedish hMID > sporadic SVD, and in basal ganglia CADASIL > HERNS > Swedish hMID > PADMAL> sporadic SVD. The subcortical white matter was almost always more affected than any grey matter. We observed glucose transporter-1 protein immunoreactivities were most affected in the white matter indicating capillary degeneration whereas collagen IV (COL4) immunostaining was increased in PADMAL cases in all regions and tissue types. Overall, GLUT-1:COL4 ratios were higher in the basal ganglia indicating modifications in capillary density compared to the frontal lobe. Our study shows the extent of microvascular degeneration varies in these genetic disorders exhibiting common end-stage pathologies but is the most aggressive in CADASIL.
Author(s): Craggs LJL, Hagel C, Kuhlenbaeumer G, Borjesson-Hanson A, Andersen O, Viitanen M, Kalimo H, McLean CA, Slade JY, Hall R, Oakley AE, Yamamoto Y, Deramecourt V, Kalaria RN
Publication type: Article
Publication status: Published
Journal: Brain Pathology
Print publication date: 01/09/2013
Online publication date: 08/03/2013
Acceptance date: 22/01/2013
ISSN (print): 1015-6305
ISSN (electronic): 1750-3639
Publisher: Wiley-Blackwell Publishing, Inc.
PubMed id: 23387519
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