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Transient Middle Cerebral Artery Occlusion Model of Neonatal Stroke in P10 Rats
07:56

Transient Middle Cerebral Artery Occlusion Model of Neonatal Stroke in P10 Rats

Published on: April 21, 2017

Siblings with infantile cerebral stroke and delayed multivessel involvement--a new hereditary vasculopathy?

Andrea Klein1, Margrit Fasnacht, Thierry A G M Huisman

  • 1Department of Neurology, University Children's Hospital Zurich, Steinwiesstrasse 75, 8032 Zürich, Switzerland. andrea.klein@kispi.unizh.ch

European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society
|April 13, 2007
PubMed

Insights

Two sisters presented with unusual stroke-like episodes and later developed systemic hypertension and narrowed arteries. This suggests a rare, inherited systemic vasculopathy affecting multiple organs.

Area of Science:

  • Neurology
  • Genetics
  • Vascular Biology

Background:

  • Investigating rare genetic disorders causing neurological deficits in children.
  • Understanding the etiology of non-atherosclerotic vasculopathies.

Observation:

  • Two sisters presented with recurrent stroke-like episodes in infancy, initially mimicking metabolic disorders.
  • Later development of pulmonary and systemic hypertension, myocardial hypertrophy, and renal artery stenosis.
  • Cranial imaging revealed moyamoya appearance with narrowed carotid and basilar arteries and collateral formation.

Findings:

  • Exclusion of metabolic stroke and MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes).
  • Systemic vasculopathy characterized by stenosis and rarefaction of pulmonary and coronary arteries.
  • Normal aorta and retinal vessels despite widespread arterial abnormalities.

Implications:

  • Suggests a novel hereditary systemic vasculopathy of unknown genetic origin.
  • Highlights the importance of long-term monitoring for systemic complications in patients with unexplained childhood strokes.
  • Underscores the need for further research into the genetic basis of rare vasculopathies.

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