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Quantitative vascular pathology and phenotyping familial and sporadic cerebral small vessel diseases

Lookup NU author(s): Dr Lucinda Craggs, Janet Slade, Roslyn Hall, Arthur Oakley, Dr Vincent Deramecourt, Professor Raj KalariaORCiD


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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.

Publication metadata

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

Year: 2013

Volume: 23

Issue: 5

Pages: 547-557

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.


DOI: 10.1111/bpa.12041

PubMed id: 23387519


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Funder referenceFunder name
Newcastle NIHR Biomedical Research Centre in Ageing and Age Related Diseases
Newcastle upon Tyne Hospitals NHS Foundation Trust
Alzheimer's Research (UK)
Alzheimer's Society
G0400074UK MRC