Neurologic syndromes in hyperammonemic disorders.

  title={Neurologic syndromes in hyperammonemic disorders.},
  author={Galen N. Breningstall},
  journal={Pediatric neurology},
  volume={2 5},
This article reviews the neurologic manifestations of hyperammonemic disorders. A variety of inborn errors of metabolism, as well as transient neonatal or acquired hepatic dysfunctions, are identified as causes of hyperammonemia. The pathogenesis of hyperammonemia in these disorders is outlined. Catastrophic hyperammonemia and hyperammonemic coma are described; algorithms to establish specific diagnosis are suggested. Symptoms of less severe ammonia intoxication, which also occur in more common… CONTINUE READING