Dtsch Med Wochenschr 2001; 126(Suppl. Gastroenterol. 1): S76-S80
DOI: 10.1055/s-2001-14502
Supplement Gastroenterologie (1)
© Georg Thieme Verlag Stuttgart · New York

Hepatische Enzephalopathie

Hepatic encephalopathyP. Ferenci
  • Universitätsklinik für Innere Medizin IV, Wien
Further Information

Publication History

Publication Date:
11 September 2002 (online)

Die hepatische Enzephalopathie (HE) ist eine metabolisch induzierte, potenziell reversible Funktionsstörung des Zentralnervensystems im Rahmen von Leberkrankheiten. Im weitesten Sinne schließt sie alle neuropsychiatrischen Syndrome, die bei akuten oder chronischen Lebererkrankungen auftreten können und kausal durch die Leberfunktionsstörung bedingt sind, ein. Die HE umfasst ein breites Spektrum unterschiedlicher klinischer Zustandsbilder. Zahlreiche Synonyme geben meist nur Teilaspekte wieder. Voraussetzung für die Diagnose einer HE ist der Nachweis einer Lebererkrankung, der Nachweis einer Funktionsstörung des zentralen Nervensystems und der sichere Ausschluss einer anderen neurologischen Erkrankung. Während der Nachweis einer Lebererkrankung bzw. die Diagnose der Funktionsstörung des zentralen Nervensystems unproblematisch ist, kann die Abgrenzung zu anderen neurologischen Erkrankungen, die bei Leberkrankheiten auftreten können, schwierig sein.

Literatur

  • 1 Amodio P, Marchetti P, Del Piccolo F. et al . Visual attention in cirrhotic patients.  Hepatology. 1998;  27 1517-1523
  • 2 Amodio P, Marchetti P, Del P iccolo F, de Tourtchaninoff M, Varghese P, Zuliani C. et al . Spectral versus visual EEG analysis in mild hepatic encephalopathy.  Clin Neurophysiol. 1999;  110 1334-1344
  • 3 Baraldi M, Zeneroli M L. Experimental hepatic encephalopathy - changes in gamma-amino-butyric acid.  Science. 1982;  216 427-431
  • 4 Barbaro G, Di Lorenzo G, Soldini M. et al . Flumazenil for hepatic encephalopathy grade III and IVa in patients with cirrhosis.  Hepatology. 1998;  28 374-378
  • 5 Basile A S, Pandl L, Jaouni T. et al . Brain concentrations of benzodiazepines are elevated in an animal model of hepatic encephalopathy.  Proc Natl Acad Sci USA. 1990;  87 5263-5267
  • 6 Basile A S, Harrison P M, Hughes R D. et al. . Relationship between plasma benzodiazepine receptor ligand concentrations and severity of hepatic encephalopathy.  Hepatology. 1994;  19 112-121
  • 7 Bucci L, Palmieri G C. Double-blind, double-dummy comparison between treatment with rifaximin and lactulose in patients with medium to severe degree hepatic encephalopathy.  Curr-Med Res Opin. 1993;  13 109-118
  • 8 Butterworth R F. The astrocytic (»peripheral-type«) benzodiazepine receptor.  Neurochem Int. 2000;  36 411-416
  • 9 Cadranel J F, el Younsi M, Pidoux B, Zylberberg P, Benhamou Y, Valla D, Opolon P. Flumazenil therapy for hepatic encephalopathy in cirrhotic patients.  Eur J Gastroenterol Hepatol. 1995;  7 325-329
  • 10 Chan H, Hazell A S, Desjardins P, Butterworth R F. Effects of ammonia on glutamate transporter (GLAST) protein and mRNA in cultured rat cortical astrocytes.  Neurochem Int. 2000;  37 243-248
  • 11 Conn H O, Leevy C M, Vlahcevic Z R, Rodgers J B, Maddrey W C, Seeff L, Levy L L. Comparison of Lactulose and Neomycin in the Treatment of Chronic Portal-systemic Encephalopathy.  Gastroenterology. 1977;  72 573-583
  • 12 Desjardins P, Rao K V, Michalak A, Rose C, Butterworth R F. Effect of portacaval anastomosis on glutamine synthetase protein and gene expression in brain, liver and skeletal muscle.  Metab Brain Dis. 1999;  14 273-280
  • 13 Egberts E H, Schomerus H, Hamster W, Jürgens P. Branched Chain Amino Acids in the Treatment of Latent Portosystemic Encephalopathy.  Gastroenterology. 1985;  88 887-895
  • 14 Efrati C, Masini A, Merli M, Valeriano V, Riggio O. Effect of sodium benzoate on blood ammonia response to oral glutamine challenge in cirrhotic patients: a note of caution.  Am J Gastroenterol. 2000;  95 3574-3578
  • 15 Ferenci P. Diagnosis and differential diagnosis of hepatic encephalopathy: Implications for the design of clinical studies. Univ. of Newcastle upon Tyne In: Advances in Hepatic encephalopathy and metabolism in liver diseases. CO Record, H Al Mardini (eds) 1997: 267-272
  • 16 Ferenci P. Hepatische Enzephalopathie: Ernährung als Ursache oder Therapie?.  Z f Gastroenterologie. 1996;  34 32-34 (Suppl 5)
  • 17 Fischer J E, Baldessarini R J. False neurotransmitters and hepatic failure.  ancet. 1971;  ii 75-80
  • 18 Grippon P, Le Poncin-Laffite M, Boschat M. et al . Evidence for the role of ammonia in the intracerebral transfer and metabolism of tryptophan.  Hepatology. 1986;  6 682-6686
  • 19 Groeneweg M, Gyr K, Amrein R, Scollo-Lavizzari G, Williams R, Yoo J Y, Schalm S W. Effect of flumazenil on the electroencephalogram of patients with portosystemic encephalopathy. Results of a double blind, randomized, placebo-controlled multicenter trial.  Electroencephalogr Clin Neurophysiol. 1996;  98 29-34
  • 20 Horst D, Grace N D, Conn H O. et al . Comparison of dietary protein with an oral, branched chain-enriched amino acid supplement in chronic portal-systemic encephalopathy.  Hepatology. 1984;  4 279-287
  • 21 Hartmann I J, Groeneweg M, Quero J C, Beijeman S J, de Man R A, Hop W C, Schalm S W. The prognostic significance of subclinical hepatic encephalopathy.  Am J Gastroenterol. 2000;  95 2029-2034
  • 22 Haussinger D, Kircheis G, Fischer R, Schliess F, vom Dahl S. Hepatic encephalopathy in chronic liver disease: A clinical manifestation of astrocyte swelling and low-grade cerebral edema?.  J Hepatol. 2000;  32 1035-1038
  • 23 Kircheis G, Nilius R, Held C. et al . Therapeutic efficacy of L-ornithine-L-aspartate infusions in patients with cirrhosis and hepatic encephalopathy: results of a placebo-controlled, double blind study.  Hepatology. 1997;  25 1351-1360
  • 24 Kramer L, Tribl B, Gendo A, Zauner C, Schneider B, Ferenci P, Madl C. Comparison of ammonia partial pressure and total ammonia in hepatic encephalopathy.  Hepatology. 2000;  31 30-34
  • 25 Krieger D, Krieger S, Jansen O, Gass P, Theilmann L, Lichtnecker H. Manganese and chronic hepatic encephalopathy.  Lancet. 1995;  346 270-274
  • 26 Kullmann F, Hollerbach S, Holstege A, Schölmerich J. Subclinical hepatic encephalopathy.  J Hepatol. 1995;  22 101-110
  • 27 Loguercio C, Abbiati R, Rinaldi M, Romano A, del Vecchio Blanco  C, Coltori M. Long-term effects of enterococcus faecium SF68 versus lactulose in the treatment of patients with cirrhosis and grade 1 - 2 hepatic encephalopathy.  J Hepatology. 1995;  23 39-46
  • 28 Marchesini G, Fabbri A, Bianchi G, Brizi M, Zoli M. Zinc supplementation and amino acid-nitrogen metabolism in patients with advanced cirrhosis.  Hepatology. 1996;  23 1084-1092
  • 29 Mendenhall C L, Rouster S, Marshall L, Weesner R. A New Therapy for Portal Systemic Encephalopathy.  Amer J Gastroenterol. 1986;  81 540-543
  • 30 Michalak A, Chatauret N, Butterworth R F. Evidence for a serotonin transporter deficit in experimental acute liver failure.  Neurochem Int. 2001;  38 163-168
  • 31 Michalak A, Rose C, Butterworth J, Butterworth R F. Neuroactive amino acids and glutamate (NMDA) receptors in frontal cortex of rats with experimental acute liver failure.  Hepatology. 1996;  24 908-913
  • 32 Morgan M Y, Hawley K E. Lactitol vs. lactulose in the treatment of acute hepatic encephalopathy in cirrhotic patients: A double blind, randomized trial.  Hepatology. 1987;  7 1278-1284
  • 33 Naylor C D, O’Rourkee K, Detsky A S, Baker J P. Parenteral nutrition with branched-chain amino acids in hepatic encephalopathy. A Meta-Analysis.  Gastroenterology. 1989;  97 1033-1042
  • 34 Orlandi F, Brunelli E, Benedetti A, Macarri G. Clinical trials of nonabsorbable disaccharide therapy in hepatic encephalopathy. Medi-Ed Press, Bloomington in: Hepatic encephalopathy. Syndromes and Therapies. Conn HO and Bircher J. (eds) 1994: 209-218
  • 35 Plauth M, Merli M, Kondrup J, Weimann A, Ferenci P, Müller M J. ESPEN Guidelines for nutrition in liver disease and transplantation.  Clin Nutrition. 1997;  16 43-55
  • 36 Pomier-Layrargues J F, Giguère J, Lavoie J. et al . Flumazenil in cirrhotic patients in hepatic coma:A randomized double-blind placebo-controlled crossover trial.  Hepatology. 1994;  19 32-37
  • 37 Quero J C, Hartmann J C, Meulstee J, Hop W C, Schalm S W. The diagnosis of subclinical encephalopathy in patients with cirrhosis using neuropsychological tests and automated electroencephalogram analysis.  Hepatology. 1996;  24 556-560
  • 38 Raabe W. Neuronal effects of ammonia. Elsevier, Amsterdam In: Advances in ammonia metabolism and hepatic encephalopathy. Soeters P, Wilson JHP, Meijer AF, Holm E (eds) 1988: 349-355
  • 39 Rees C J, Oppong K, Al Mardini  H, Hudson M, Record C O. Effect of L-ornithine-L-aspartate on patients with and without TIPS undergoing glutamine challenge: a double blind, placebo controlled trial.  Gut. 2000;  47 571-574
  • 40 Reding P, Duchateau J, Bataille C. Oral zinc supplementation improves hepatic encephalopathy.   Lancet. 1984;  ii 493-495
  • 41 Riggio O, Ariosto F. et al . Short-term oral zinc supplementation does not improve chronic hepatic encephalopathy.  Dig Dis Sci. 1991;  36 1204-1208
  • 42 Ross B D, Jacobson S, Villamil F, Korula J, Kreis R, Ernst T. et al . Subclinical hepatic encephalopathy.  Radiology. 1994;  193 457-463
  • 43 Rudman D, Smith R B, Salam A A. et al . The amino acid content of food.  Am J Clin Nutr. 1973;  26 487-490
  • 44 Staedt U, Leweling H, Gladisch R, Kortsik C, Hagmuller E, Holm E. Effects of ornithine aspartate on plasma ammonia and plasma amino acids in patients with cirrhosis.   J Hepatol. 1993;  19 424-430
  • 45 Strauss E, Tramote R, Silva E P, Caly W R, Honain N Z, Maffei R A, de Sa M F. Double-blind randomized clinical trial comparing neomycin and placebo in the treatment of exogenous hepatic encephalopathy.  Hepatogastroenterology. 1992;  39 542-545
  • 46 Sushma S, Dasarathy S, Tandon R K, Jain S, Gupta S, Bhist M S. Sodium benzoate in the treatment of acute hepatic encephalopathy: a double-blind randomized trial.  Hepatology. 1992;  16 138-144
  • 47 Tarao K, Ikeda T, Hayashi K, Sakurai A, Okada T, Ito T. et al . Successful use of vancomycin hydrochloride in the treatment of lactulose resistant chronic hepatic encephalopathy.  Gut. 1990;  31 702-706
  • 48 Tromm A, Griga T, Greving I, Hilden H, Huppe D, Schwegler U. et al . Orthograde whole gut irrigation with mannite versus paromomycine + lactulose as prophylaxis of hepatic encephalopathy in patients with cirrhosis and upper gastrointestinal bleeding: Results of a controlled randomized trial.  Hepatogastroenterology. 2000;  47 473-477
  • 49 Uribe M, Campollo O, Vargas F. Acidifying enemas (lactitol and lactulose) vs. nonacidifying enemas (tap water) to treat acute portal-systemic encephalopathy: A double-blind randomized clinical trial.  Hepatology. 1987;  7 639-634
  • 50 Uribe M, Marquez M A, Gargaramos G. et al . Treatment of chronic portal systemic encephalopathy with lactose in lactase deficient patients.  Dig Dis Sci. 1980;  25 914-928
  • 51 Van der Rijt C C, Schalm S W, Meulstee J, Stijnen T. Flumazenil therapy for hepatic encephalopathy. A double-blind cross over study.  Gastroenterol Clin Biol. 1995;  19 572-580
  • 52 Van der Rijt C C, Schalm S W, Schat H, Foeken K, De Jong G. Overt hepatic encephalopathy precipitated by zinc deficiency.  Gastroenterology. 1991;  100 1114-1118
  • 53 Vogels B APM, Maas M AW, Daalhuisen J, Quack G, Chamuleau R AFM. Memantine, a noncompetitive NMDA receptor antagonist improves hyperammonemia-induced encephalopathy and acute hepatic encephalopathy in rats.  Hepatology. 1997;  25 820-827
  • 54 Weber F L, Minco D, Fresard K M, Banwell J G. Effects of vegetable diets on nitrogen metabolism in cirrhotic subjects.  Gastroenterology. 1985;  89 538-544
  • 55 Weissenborn K, Rückert N, Hecker H, Manns M P. The number connection tests A and B: Interindividual variability and use for the assessment of early hepatic encephalopathy.  J Hepatol. 1998;  28 646-653
  • 56 Wiltfang J, Nolte W, Otto M, Wildberg J, Bahn E, Figulla H R. et al . Elevated serum levels of astroglial S100beta in patients with liver cirrhosis indicate early and subclinical portal-systemic encephalopathy.  Metab Brain Dis. 1999;  14 239-251
  • 57 Yurdaydin C, Herneth A, Steindl P, Singer E, Ferenci P. Modulation of hepatic encephalopathy in rats with thioacetamide-induced acute liver failure by serotonin antagonists.  Eur J Gastroenterol Hepatol. 1996;  8 664-671
  • 58 Yurdaydin C, Hörtnagl H, Steindl P, Zimmermann C, Pifl C, Singer E A, Roth E, Ferenci P. Increased serotoninergic and noradrenergic activity in hepatic encephalopathy in rats with thioacetamide induced acute liver failure.  Hepatology. 1990;  12 695-700

Univ. Prof. Dr. Peter Ferenci

Universitätsklinik für Innere Medizin IV Klinische Abteilung Gastroenterologie und Hepatologie

Währinger Gürtel 18-20

A-1090 Wien

Email: peter.ferenci@akh-wien.ac.at