Skip to main content

Acute Metabolic Encephalopathy: A Review of Causes, Mechanisms and Treatment

  • Chapter

Summary

Acute encephalopathy is a relatively common problem: one of the causes is metabolic disorders. A detailed history, examination and investigations performed during the acute illness (blood sugar, blood gases, plasma ammonia, blood lactate, plasma ketones, plasma amino acids, liver function tests, and urinary organic acids) should identify those patients in whom a metabolic disorder is likely. More detailed studies may be needed to establish a precise diagnosis.

The mechanism of the acute brain dysfunction is multifactorial. Factors that contribute include changes in blood flow and, initially, a disturbance in neurotransmitter function followed by failure of energy metabolism and cellular depolarization. Treatment of these conditions is largely supportive, with especial attention to the management of cerebral perfusion pressure.

This is a preview of subscription content, log in via an institution.

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   39.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  • Ahmed, K. and Schofield, P. G. Studies on fatty acid oxidation 8. The effects of fatty acids on metabolism of rat brain cortex in vitro. Biochem. J. 81 (1961) 45–53

    CAS  Google Scholar 

  • Alger, B. E. and Nicoll, R. A. Ammonia does not selectively block IPSPs in rat hippocampal pyramidal cells. J. Neurophysiol. 49 (1983) 1381–1391

    PubMed  CAS  Google Scholar 

  • Aynsley-Green, A. and Soltesz, G. Hypoglycaemia in infancy and childhood. Current Reviews in Paediatrics, Vol. 1, Churchill Livingston, Edinburgh and London, 1985, pp. 151–161

    Google Scholar 

  • Batshaw, M. L., Walser, M. and Brusilow, S. Plasma u-ketoglutarate in urea cycle enzymopathies and its role as harbinger of hyperammonemic coma. Pediatr. Res. 14 (1980) 1316–1319

    Article  PubMed  CAS  Google Scholar 

  • Bessman, S. P. and Bessman, A. N. The cerebral and peripheral uptake of ammonia in liver disease with an hypothesis for the mechanism of hepatic coma. J. Clin. Invest. 34 (1955) 622–628

    Article  PubMed  CAS  Google Scholar 

  • de Bont, B., Moulin, D., Stein, F., van Hoof, F. and Lauwerys, R. Peritoneal dialysis with o-penicillamine in Wilson disease. J. Pediatr. 107 (1985) 545–547

    Article  PubMed  Google Scholar 

  • Bradford, H. F. and Ward, H. K. On glutaminase activity in mammalian synaptosomes. Brain. Res. 110 (1976) 115–125

    Article  PubMed  CAS  Google Scholar 

  • Brusilow, S. W. Inborn errors of urea synthesis. In Lloyd, J. K. and Scriver, C. R. (Eds.), Genetic and Metabolic Disease in Paediatrics, Butterworths, London, 1985, pp. 140–165

    Google Scholar 

  • Chapman, A. G., Westerberg, E. and Siesjö, B. K. The metabolism of purine and pyrimidine nucleotides in rat cortex during insulin-induced hypoglycaemia and recovery. J. Neurochem. 36 (1981) 179–189

    Article  PubMed  CAS  Google Scholar 

  • van Erven, P. M. M., Cillesen, J. P. M., Eekhoff, E. M. W., Gabreëls, F. J. M., Doesberg, W. H., Lemmens, W. A. J. G., Slooff, J. L., Renier, W. O. and Ruitenbeek, W. Leigh syndrome, a mitochondrial encephalo(myo)opathy. Clin. Neurol. Neurosurg. 89 (1987) 217–230

    Article  PubMed  Google Scholar 

  • Fraser, C. L. and Arieff, A. I. Hepatic encephalopathy. N. Engl. J. Med. 313 (1985) 865–873

    Article  PubMed  CAS  Google Scholar 

  • Ghajar, J. B. G., Plum, F. and Duffy, T. E. Cerebral oxidative metabolism and blood flow during acute hypoglycaemia and recovery in unanaesthetised rats. J. Neurochem. 38 (1982) 397–409

    Article  PubMed  CAS  Google Scholar 

  • Gabreëls, F. J. M., Prick, M. J. J., Trijbels, J. M. F., Renier, W. O., Jaspar, H. H. J.. Jansen, A. J. M. and Sloof, J. L. Defects in citric acid cycle and the electron transport chain in progressive poliodystrophy. Acta Neurol. Scand. 70 (1984) 145–154

    Article  PubMed  Google Scholar 

  • Gibson, G. E. and Blass, J. P. Impaired synthesis of acetylcholine in brain accompanying mild hypoxia and hypoglycaemia. J. Neurochem. 27 (1976) 37–42

    Article  PubMed  CAS  Google Scholar 

  • Hindfield, B., Plum, F. and Duffy, T. E. Effect of acute ammonia intoxication on cerebral metabolism in rats with portocaval shunts. J. Clin. Invest. 59 (1977) 386–396

    Article  Google Scholar 

  • Kendall, B. E., Kingsley, D. P. E., Leonard, J. V., Lingam, S. and Oberholzer, V. G. Neurological features and computed tomography of the brain in children with ornithine carbamoyl transferase deficiency. J. Neurol. Neurosurg. Psychiatr. 46 (1983) 28–34

    Article  PubMed  CAS  Google Scholar 

  • Koh, T. H. H. G., Aynsley-Green, A., Tarbit, M. and Eyre, J. A. Neural dysfunction during hypoglycaemia. Arch. Dis. Child. 63 (1988) 1353–1358

    Article  PubMed  CAS  Google Scholar 

  • Leonard, J. V. Hyperammonaemia in childhood. In Clayton, B. E. and Round, J. M. (Eds.), Chemical Pathology and the Sick Child, Blackwell, Oxford, 1984, pp. 96–119

    Google Scholar 

  • Leonard, J. V. The early detection and management of inborn errors presenting acutely in the neonatal period. Eur. J. Pediatr. 143 (1983) 253–257

    Article  Google Scholar 

  • Leonard, J. V. and Dunger, D. B. Hypoglycaemia complicating feeding regimes for glycogen storage disease. Lancet 2 (1978) 1203–1204

    Article  PubMed  CAS  Google Scholar 

  • Lewis, L. D., Ljundgren, B., Ratcheson, R. A. and Siesjö, B. K. Changes in the carbohydrate substrates, amino acids and ammonia in the brain during insulin-induced hypoglycaemia. J. Neurochem. 23 (1974) 659–671

    Article  PubMed  CAS  Google Scholar 

  • Lopez-Lahoya, J., Garcia, M. L., Benavides, J. and Ugarte, M. Inhibition by methylmalonate of glycine uptake by synaptosomes from the rat spinal cord. J. Neurochem. 36 (1981) 325–327

    Article  PubMed  CAS  Google Scholar 

  • McCandless, D. W. and Schenker, S. Effects of acute ammonia intoxication on energy stores in the reticular activating system Exp. Brain Res. 44 (1981) 325–330

    Article  PubMed  CAS  Google Scholar 

  • Mans, A., Biebuyk, J. and Hawkins, R. Brain tryptophan abnormalities in hyperammonaernia and liver disease. In Bender, D. A., Joseph, M. H., Kochen, W. and Steinhart, H. (Eds.), Progress in Tryptophan and Serotonin Research 1986, Walter de Guyter, Berlin, 1987, pp. 207–213

    Google Scholar 

  • Martin, J. J. and Schlote, W. Central nervous system lesions in disorders of amino-acid metabolism: a neuropathological study. J. Neurol. Sci. 15 (1972) 49–76

    Article  PubMed  CAS  Google Scholar 

  • Montagna, P., Gallassi, R., Medori, R., Govone, E., Eeviani, M., di Mauro, S., Lugaresi, E. and Andermann, F. MELAS syndrome: characteristic migrainous and epileptic features and maternal transmission. Neurology 38 (1988) 751–754

    PubMed  CAS  Google Scholar 

  • Norberg, K., Siesjö, B. K. Oxidative metabolism of the cerebral cortex of the rat in severe insulin-induced hypoglycaemia. J. Neurochem. 26 (1976) 179–189

    Google Scholar 

  • Partin, J. C., Bove, K., Partin, J. S. and Schubert, W. K. Liver and muscle ultrastructure in Reye’s syndrome. In Crocker, J. F. S. (Ed.), Reye’s Syndrome, Vol. II Grune and Stratton, New York, 1979, pp. 215–232

    Google Scholar 

  • Pincus, J. H. Subacute necrotising encephalomyelopathy (Leigh’s disease): a consideration of clinical features and etiology. Dev. Med. Child. Neurol. 14 (1972) 87–101

    Article  PubMed  CAS  Google Scholar 

  • della Porta, P., Maiolo, A. T., Negri, V. U. and Rosella, E. Cerebral blood flow and metabolism in therapeutic insulin coma. Metabolism 13 (1964) 131–140

    Article  PubMed  CAS  Google Scholar 

  • Raabe, W. Synaptic transmission in ammonia intoxication. Neurochem. Pathol. 67 (1987) 145–166

    Article  Google Scholar 

  • Raabe, W. and Lin, S. Pathophysiology of ammonia intoxification. Exp. Neurol. 87 (1985) 519–532

    Article  PubMed  CAS  Google Scholar 

  • Russell, G. J., Fitzgerald, J. F. and Clark, J. H. Fulminant hepatic failure. J. Pediatr. 111 (1987) 313–319

    Article  PubMed  CAS  Google Scholar 

  • Saudubray, J. M., Marsac, C., Limal, J. M., Dumurgier, E., Charpentier, C., Ogier, H. and Coudè, F. X. Variation in plasma ketone bodies during a 24-hour fast in normal and in hypoglycaemic children: relationship to age. J. Pediatr. 98 (1981) 904–908

    Article  PubMed  CAS  Google Scholar 

  • Saudubray, J. M., Specola, N., Middleton, B., Lombes, A., Bonnefont, J. P., Jakobs, C., Vassault, A., Charpentier, C. and Day, R. Hyperketotic states due to inherited defects of ketolysis. Enzyme 38 (1987) 80–90

    PubMed  CAS  Google Scholar 

  • Siesjö, B. K. Hypoglycaemia, brain metabolism, and brain damage. Diabetes Metab. Rev. 4 (1988) 113–144

    Article  PubMed  Google Scholar 

  • Siesjö, B. K. and Plum, F. Pathophysiology of anoxic brain damage. In Gaull, G. E. (Ed.), Biology of Brain Dysfunction, Plenum Press, New York, 1972, pp. 319–372

    Google Scholar 

  • Sokol, R. J., Francis, P. D., Gold, S. H., Ford, D. M., Lum, G. M. and Ambruso, D. R. Orthotopic liver transplantation for acute fulminant Wilson disease. J. Pediatr. 107 (1985) 549–552

    Article  PubMed  CAS  Google Scholar 

  • Stanbury, J. B., Wyngaarden, J. B., Fredrickson, D. S., Goldstein, J. L. and Brown, M. S. (Eds.). The Metabolic Basis of Inherited Disease, 5th edn., McGraw-Hill Book Co., New York, 1983

    Google Scholar 

  • Tashian, R. E. Inhibition of brain glutamic dehydrogenase by phenylalanine, leucine and valine derivatives: a suggestion concerning the neurological defect in phenylketonuria and branched-chain ketoaciduria. Metabolism 10 (1961) 393–402

    PubMed  CAS  Google Scholar 

  • Voorhies, T. M., Ehrlich, M. E., Duffy, T. E., Petito, C. K. and Plum, F. Acute hyperammonaemia in the young primate: physiologic and neuropathologic correlates. Pediatr. Res. 17 (1983) 970–975

    Article  PubMed  CAS  Google Scholar 

  • Walker, C. O., McCandless, D. W., McGarry, J. D. and Shenker, S. Cerebral energy metabolism in short chain fatty acid induced coma. J. Lab. Clin. Med. 76 (1970) 569–583

    PubMed  CAS  Google Scholar 

  • Walter, J. H. and Leonard, J. V. Inborn errors of the urea cycle. Br. J. Hosp. Med. 38 (1987) 176–183

    PubMed  CAS  Google Scholar 

  • Ware, A. J., d’Agostino, A. and Combes, B. Cerebral oedema: a major complication of massive hepatic necrosis. Gastroenterology 76 (1979) 123–131

    Google Scholar 

  • Watson, A. J., Karp, J. E., Walker, W. G., Chamber, T., Risch, V. R. and Brusilow, S. W. Transient idiopathic hyperammonaemia in adults. Lancet 2 (1985) 1271–1274

    Article  PubMed  CAS  Google Scholar 

  • Wollff, J. A., Caroll, J. E., Thuy, L. P., Haas, R. and Nyhan, W. L. Carnitine reduces fasting ketogenesis in patients with disorders of propionate metabolism. Lancet 1 (1986) 289–291

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

G. M. Addison J. M. Connor R. A. Harkness R. J. Pollitt

Rights and permissions

Reprints and permissions

Copyright information

© 1989 SSIEM and Kluwer Academic Publishers

About this chapter

Cite this chapter

Surtees, R., Leonard, J.V. (1989). Acute Metabolic Encephalopathy: A Review of Causes, Mechanisms and Treatment. In: Addison, G.M., Connor, J.M., Harkness, R.A., Pollitt, R.J. (eds) Studies in Inherited Metabolic Disease. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-1069-0_5

Download citation

  • DOI: https://doi.org/10.1007/978-94-009-1069-0_5

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-010-6970-0

  • Online ISBN: 978-94-009-1069-0

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics