Acute hyperammonemic encephalopathy

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Acute hyperammonemic encephalopathy. Organic acidaemias, Citrullinaemia. Fully referenced neurology checklist.

Metabolic causes

  • Urea cycle disorders e.g. ornithine transcarbamylase deficiency (OTC)
  • Organic acidaemias
  • Citrullinaemia

Systemic causes

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Drug-induced

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Clinical features

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Magnetic resonance imaging (MRI) brain

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Treatment: metabolic

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Treatment: anti-epileptic drugs (AEDs)

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References

  1. U-King-Im JM, Yu E, Bartlett E, Soobrah R, Kucharczyk W. Acute hyperammonemic encephalopathy in adults: imaging findings. Am J Neuroradiol 2011; 32:413-418.
  2. Takanashi J, Barkovich AJ, Cheng SF, Kostiner D, Baker JC, Packman S. Brain MR imaging in acute hyperammonemic encephalopathy arising from late-onset ornithine transcarbamylase deficiency. Am J Neuroradiol 2003; 24:390-393.
  3. Madathil RJ, Gilstrap LG, Pelletier MP, Mehra MR. Isolated hyperammonemic encephalopathy in heart transplantation. J Heart Lung Transplant 2018; 37:427-429.
  4. López-Blanco R, de Fuenmayor-Fernández de la Hoz CP, González de la Aleja J, Martínez-Sánchez P, Ruiz-Morales J. Hyperammonemic encephalopathy associated with multiple myeloma. Neurologia 2017; 32:339-341.
  5. Lewis C, Deshpande A, Tesar GE, Dale R. Valproate-induced hyperammonemic encephalopathy: a brief review. Curr Med Res Opin 2012; 28:1039-1042.

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