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A Volumetric Method for Quantification of Cerebral Vasospasm in a Murine Model of Subarachnoid Hemorrhage
08:12

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Published on: July 28, 2018

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

Lucinda J L Craggs1, Christian Hagel, Gregor Kuhlenbaeumer

  • 1Centre for Brain Ageing and Vitality, Institute for Ageing and Health, Newcastle University, Newcastle upon Tyne, UK.

Brain Pathology (Zurich, Switzerland)
|February 8, 2013
PubMed

Insights

Vascular pathology in inherited small vessel diseases (SVDs) varies, with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) showing the most severe microvascular degeneration. This study compared four genetic SVDs, finding CADASIL most aggressive.

Area of Science:

  • Neurology
  • Genetics
  • Pathology

Background:

  • Inherited small vessel diseases (SVDs) share common end-stage pathologies but exhibit varying clinical presentations.
  • Understanding the specific microvascular changes in different genetic SVDs is crucial for diagnosis and treatment.

Purpose of the Study:

  • To quantify and compare vascular pathology in the frontal lobe and basal ganglia across four distinct inherited SVDs.
  • To investigate differences in microvascular degeneration and protein expression (GLUT-1, COL4) among these genetic disorders.

Main Methods:

  • Histopathological analysis of frontal lobe and basal ganglia tissue from patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), pontine autosomal dominant microangiopathy and leukoencephalopathy (PADMAL), hereditary multi-infarct dementia of Swedish type (Swedish hMID), and hereditary endotheliopathy with retinopathy, nephropathy, and stroke (HERNS).
  • Quantification of vascular pathology using sclerotic index.
  • Immunohistochemical staining for glucose transporter-1 (GLUT-1) and collagen IV (COL4) to assess capillary integrity and density.

Main Results:

  • Vascular pathology was most severe in CADASIL, with greater severity observed in the basal ganglia compared to the frontal lobe.
  • The order of vascular pathology severity in the frontal lobe was CADASIL ≥ HERNS > PADMAL > Swedish hMID > sporadic SVD.
  • Subcortical white matter was consistently more affected than gray matter; GLUT-1 was reduced in white matter, while COL4 increased in PADMAL cases.

Conclusions:

  • The extent of microvascular degeneration differs significantly among inherited SVDs, despite shared end-stage pathologies.
  • CADASIL demonstrates the most aggressive microvascular changes among the studied genetic disorders.
  • Variations in GLUT-1:COL4 ratios suggest region-specific modifications in capillary density within these SVDs.

Keywords:
CADASILHERNSPADMALhereditary multi-infarct dementiasclerotic indexsmall vessel disease

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