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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
 

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Extrapontine myelinolysis resulting in transient cortical blindness.

Central pontine myelinolysis and extrapontine myelinolysis are characterized by symmetric demyelination subsequent to rapid shifts in serum osmolality. Described here is a novel case of transient cortical blindness in association with imaging features of extrapontine myelinolysis, which occurred in a child with carbamoyl phosphate synthetase deficiency after rapid correction of hyperammonemia. Serum sodium levels were within normal limits at presentation and throughout the period of ammonia correction. A potential pathogenic mechanism of osmotic demyelination in the setting of acute treatment for hyperammonemia in a patient with a urea cycle abnormality includes disruption of the blood-brain barrier and re-equilibration of organic osmolytes, particularly glutamine.[1]

References

  1. Extrapontine myelinolysis resulting in transient cortical blindness. Langer, J.E., Wilson, W.G., Raghavan, P., Rust, R.S., Goodkin, H.P. Pediatr. Neurol. (2010) [Pubmed]
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