Cerebral haemodynamics in patients with glutaryl-coenzyme A dehydrogenase deficiency.

@article{Strauss2010CerebralHI,
  title={Cerebral haemodynamics in patients with glutaryl-coenzyme A dehydrogenase deficiency.},
  author={Kevin A Strauss and Patrick Donnelly and Max Wintermark},
  journal={Brain : a journal of neurology},
  year={2010},
  volume={133 Pt 1},
  pages={76-92}
}
In glutaric aciduria type 1, glutaryl-coenzyme A and its derivatives are produced from intracerebral lysine and entrapped at high concentrations within the brain, where they interfere with energy metabolism. Biochemical toxicity is thought to trigger stroke-like striatal degeneration in susceptible children under 2 years of age. Here, we explore vascular derangements that might also contribute to brain damage. We studied injured and non-injured Amish glutaric aciduria type 1 patients using… CONTINUE READING
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