Portosystemic encephalopathy

Handbook of Clinical Neurology

Volumn 120, Issue , 2014, Pages 661-674

  Weissenborn, Karin ^a  

^a HANNOVER MEDICAL SCHOOL   (Germany)

Author keywords

Clinical features; Differential diagnosis; Hepatic encephalopathy; Minimal hepatic encephalopathy; Therapy

Indexed keywords

ARTICLE; CLINICAL FEATURE; DIFFERENTIAL DIAGNOSIS; ELECTROENCEPHALOGRAPHY; HEPATIC ENCEPHALOPATHY; HISTORY; HUMAN; MINIMAL HEPATIC ENCEPHALOPATHY; NUCLEAR MAGNETIC RESONANCE IMAGING; THERAPY;

CLINICAL FEATURES; DIFFERENTIAL DIAGNOSIS; HEPATIC ENCEPHALOPATHY; MINIMAL HEPATIC ENCEPHALOPATHY; THERAPY;

DIAGNOSIS, DIFFERENTIAL; ELECTROENCEPHALOGRAPHY; HEPATIC ENCEPHALOPATHY; HISTORY, 19TH CENTURY; HUMANS; MAGNETIC RESONANCE IMAGING;

EID: 84890841590     PISSN: 00729752     EISSN: None     Source Type: Book Series    
DOI: 10.1016/B978-0-7020-4087-0.00045-0     Document Type: Chapter

References (116)

1. Adams R.D., Foley J.M. The neurological disorders associated with liver disease. Proc Assoc Res Nerv Ment Dis 1952, 32:198-237.
2. Ahboucha S., Butterworth R.F. The neurosteroid system: implication in the pathophysiology of hepatic encephalopathy. Neurochem Int 2007, 52:575-587.
3. Ahl B., Weissenborn K., van den Hoff J., et al. Regional differences in cerebral blood flow and cerebral ammonia metabolism in patients with cirrhosis. Hepatology 2004, 40:73-79.
4. Als-Nielsen B., Gluud L.L., Gluud C. Non-absorbable disaccharides for hepatic encephalopathy: systematic review of randomised trials. BMJ 2004, 328:1046-1051.
5. Amodio P., Campagna F., Olianas S., et al. Detection of minimal hepatic encephalopathy: normalization and optimization of the Psychometric Hepatic Encephalopathy Score. A neuropsychological and quantified EEG study. J Hepatol 2008, 49:346-353.
6. Amodio P., Ridola L., Schiff S., et al. Improving detection of minimal hepatic encephalopathy using the inhibitory control task. Gastroenterology 2010, 139:510-518.
7. Angeli P., Wong F., Watson H., et al. Hyponatremia in cirrhosis: results of a patient population survey. Hepatology 2006, 44:1535-1542.
8. Aschner M., Guilarte T.R., Schneider J.S., et al. Manganese: recent advances in understanding its transport and neurotoxicity. Toxicol Appl Pharmacol 2007, 221:131-147.
9. Bajaj J.S., Saeian K., Verber M.D., et al. Inhibitory control test is a simple method to diagnose minimal hepatic encephalopathy and predict development of overt hepatic encephalopathy. Am J Gastroenterol 2007, 102:754-760.
10. Bajaj J.S., Saeian K., Schubert C.M., et al. Minimal hepatic encephalopathy is associated with motor vehicle crashes: the reality beyond the driving test. Hepatology 2009, 50:1175-1183.
11. Balata S., Olde Damink S.W.M., Ferguson K., et al. Induced hyperammonemia alters neuropsychology, brain MR spectroscopy and magnetization transfer in cirrhosis. Hepatology 2003, 37:931-939.
12. Bass N.M., Mullen K.D., Sanyal A., et al. Rifaximin treatment in hepatic encephalopathy. N Engl J Med 2010, 362:1071-1081.
13. Burkhard P.R., Delavelle J., Du Pasquier R., et al. Chronic parkinsonism associated with cirrhosis: a distinct subset of acquired hepatocerebral degeneration. Arch Neurol 2003, 60:521-528.
14. Butterworth R.F. Pathophysiology of hepatic encephalopathy: a new look at ammonia. Metab Brain Dis 2002, 17:221-227.
15. Butterworth R.F. Neuronal cell death in hepatic encephalopathy. Metab Brain Dis 2007, 22:309-320.
16. Butterworth R.F., Spahr L., Fontaine S., et al. Manganese toxicity, dopaminergic dysfunction and hepatic encephalopathy. Metab Brain Dis 1995, 10:259. 227.
17. Cadranel J.-F., Lebiez E., Di Martino V., et al. Focal neurological signs in hepatic encephalopathy in cirrhotic patients: an underestimated entity?. Am J Gastroenterol 2001, 96:515-518.
18. Cagnin A., Taylor-Robinson S., Forton D.M., et al. In vivo imaging of cerebral "peripheral benzodiazepine binding sites" in patients with hepatic encephalopathy. Gut 2006, 55:547-553.
19. Campellone J.V., Lacomis D., Giuliani M.J., et al. Hepatic myelopathy. Case report with review of the literature. Clin Neurol Neurosurg 1996, 98:242-246.
20. Cash W.J., McConville P., McDermott E., et al. Current concepts in the assessment and treatment of hepatic encephalopathy. Q J Med 2010, 103:9-16.
21. Conn H.O., Lieberthal M.M. The Hepatic Coma Syndromes and Lactulose 1979, Williams and Wilkins, Baltimore.
22. Cordoba J., Garcia-Martinez R., Simon-Talero M. Hyponatremic and hepatic encephalopathies: similarities, differences and coexistence. Metab Brain Dis 2010, 25:73-80.
23. Davies M.G., Rowan M.J., Feely J. EEG and event related potentials in hepatic encephalopathy. Metab Brain Dis 1991, 6:175-186.
24. Da Rocha, Braga F.T., da Silva C.J., et al. Reversal of parkinsonism and portosystemic encephalopathy following embolization of a congenital intrahepatic venous shunt: brain MR imaging and 1H spectroscopic findings. AJNR 2004, 25:1247-1250.
25. Dhiman R.K., Kurmi R., Thumburu K.K., et al. Diagnosis and prognostic significance of minimal hepatic encephalopathy in patients with cirrhosis of liver. Dig Dis Sci 2010, 55:2381-2390.
26. Ferenci P., Lockwood A., Mullen K., et al. Hepatic encephalopathy - definition, nomenclature, diagnosis, and quantification: final report of the working party at the 11th World Congresses of Gastroenterology, Vienna, 1998. Hepatology 2002, 35:716-721.
27. Ferrara J., Jankovic J. Acquired hepatocerebral degeneration. J Neurol 2009, 256:320-332.
28. Fichet J., Mercier E., Genee O., et al. Prognosis and 1-year mortality of intensive care unit patients with severe hepatic encephalopathy. J Crit Care 2009, 24:364-370.
29. Giewekemeyer K., Berding G., Ahl B., et al. Bradykinesia in cirrhotic patients with early hepatic encephalopathy is related to a decreased glucose uptake of frontomesial cortical areas relevant for movement initiation. J Hepatol 2007, 46:1034-1039.
30. Gilberstadt S.J., Gilberstadt H., Zieve L., et al. Psychomotor performance defects in cirrhotic patients without overt encephalopathy. Arch Intern Med 1980, 140:519-521.
31. Goldbecker A., Buchert R., Berding G., et al. Blood brain barrier permeability for ammonia in patients with different grades of liver fibrosis and normal plasma ammonia levels is not different from healthy controls. J Cereb Blood Flow Metab 2010, 30:1384-1393.
32. Görg B., Qvartskhava N., Keitel V., et al. Ammonia induces RNA oxidation in cultured astrocytes and brain in vivo. Hepatology 2008, 48:567-579.
33. Görg B., Qvartskhava N., Bidmon H.-J., et al. Oxidative stress markers in the brain of patients with cirrhosis and hepatic encephalopathy. Hepatology 2010, 52:256-265.
34. Guerit J.M., Amantini A., Fischer C., et al. Neurophysiological investigations of hepatic encephalopathy: ISHEN practice guidelines. Liver Int 2009, 29:789-796.
35. Guevara M., Baccaro M.E., Torre A., et al. Hyponatremia is a risk factor of hepatic encephalopathy in patients with cirrhosis: a prospective study with time-dependent analysis. Am J Gastroenterol 2009, 104:1382-1389.
36. Häussinger D., Schliess F. Pathogenetic mechanisms of hepatic encephalopathy. Gut 2008, 57:1156-1165.
37. Häussinger D., Kircheis G., Fischer R., et al. Hepatic encephalopathy in chronic liver disease: a clinical manifestation of astrocyte swelling and low-grade cerebral edema?. J Hepatol 2000, 32:1035-1038.
38. Jayakumar A.R., Rama Rao K.V., Kalaiselvi P., et al. Combined effects of ammonia and manganese on astrocytes in culture. Neurochem Res 2004, 29:2051-2056.
39. Jepsen P., Ott P., Andersen P.K., et al. Clinical course of alcoholic liver cirrhosis: a Danish population-based cohort study. Hepatology 2010, 51:1675-1682.
40. Kale R.A., Gupta R.K., Saraswat V.A., et al. Demonstration of interstitial cerebral edema with diffusion tensor MR imaging in type C hepatic encephalopathy. Hepatology 2006, 43:698-706.
41. Keiding S., Sørensen M., Bender D., et al. Brain metabolism of 13N-ammonia during acute hepatic encephalopathy in cirrhosis measured by positron emission tomography. Hepatology 2006, 43:42-50.
42. 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.
43. Kircheis G., Wettstein M., Timmermann L., et al. Critical flicker frequency for quantification of low-grade hepatic encephalopathy. Hepatology 2002, 35:357-366.
44. Kircheis G., Knoche A., Hilger N., et al. Hepatic encephalopathy and fitness to drive. Gastroenterology 2009, 137:1706-1715.
45. Krieger S., Jauß M., Jansen O., et al. Neuropsychiatric profile and hyperintense globus pallidus on T1-weighted magnetic resonance images in liver cirrhosis. Gastroenterology 1996, 111:147-155.
46. Kril J.J., Butterworth R.F. Diencephalic and cerebellar pathology in alcoholic and nonalcoholic patients with end-stage liver disease. Hepatology 1997, 26:837-841.
47. Köstler H. Proton magnetic resonance spectroscopy in portal-systemic encephalopathy. Metab Brain Dis 1998, 13:291-301.
48. Kumar R., Gupta R.K., Elderkin-Thompson V., et al. Voxel-based diffusion tensor magnetic resonance imaging evaluation of lowgrade hepatic encephalopathy. J Magn Reson Imaging 2008, 27:1061-1068.
49. Leevy C.B., Phillips J.A. Hospitalizations during the use of rifaximin versus lactulose for the treatment of hepatic encephalopathy. Dig Dis Sci 2007, 52:737-741.
50. Leonhardt H., Bungert H.J. Therapie der schweren Hyper ammoniamie. Med Klin 1972, 67:1052-1056.
51. Lewis M.B., MacQillan G., Bamford J.M., Howdle P.D. Delayed myelopathic presentation of the acquired hepatocerebral degeneration syndrome. Neurology 2000, 54:1011.
52. Lockwood A.H., Yap E.W.H., Wong W.-H. Cerebral ammonia metabolism in patients with severe liver disease and minimal hepatic encephalopathy. J Cereb Blood Flow Metab 1991, 11:337-341.
53. Lockwood A.H., Murphy B.W., Donnelly K.Z., et al. Positron emission tomographic localization of abnormalities of brain metabolism in patients with minimal hepatic encephalopathy. Hepatology 1993, 18:1061-1068.
54. Lockwood A.H., Weissenborn K., Butterworth R.F. An image of the brain in patients with liver disease. Curr Opin Neurol 1997, 10:525-533.
55. Lockwood A.H., Weissenborn K., Bokemeyer M., et al. Correlations between cerebral glucose metabolism and neuropsychological test performance in non-alcoholic cirrhotics. Metab Brain Dis 2002, 17:29-40.
56. Marchesini G., Bianchi G., Merli M., et al. Nutritional supplementation with branched-chain amino acids in advanced cirrhosis: a double-blind, randomized trial. Gastroenterology 2003, 124:1792-1801.
57. Marks M.E., Jackson C.D., Montagnese S., et al. Derivation of a normative UK database for the psychometric hepatic encephalopathy score (PHES): confounding effect of ethnicity and test scoring. J Hepatol 2008, 48:S119.
58. Martin H., Voss K., Hufnagl P., et al. Morphometric and densitometric investigations of protoplasmic astrocytes and neurons in human hepatic encephalopathy. Exp Pathol 1987, 32:241-250.
59. Montagnese S., Amodio P., Morgan M.Y. Methods for diagnosing hepatic encephalopathy in patients with cirrhosis: a multidimensional approach. Metab Brain Dis 2004, 19:281-312.
60. Montagnese S., Jackson C., Morgan M.Y. Spatio-temporal decomposition of the electroencephalogram in patients with cirrhosis. J Hepatol. 2007, 46(3):447-458.
61. Mookerjee R., Stadlbauer V., Lidder S., et al. Neutrophil dysfunction in alcoholic hepatitis superimposed on cirrhosis is reversible and predicts outcome. Hepatology 2007, 46:831-840.
62. Mooney S., Hasssanein T.I., Hilsabeck R.C., et al. The UCSD Hepatology Neurobehavioral Research Program. Utility of the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) in patients with end-stage liver disease awaiting liver transplant. Arch Clin Neuropsychol 2007, 22:175-186.
63. Morgan M.Y., Hawley K.E. Lactitol versus lactulose in the treatment of acute hepatic encephalopathy in cirrhotic patients: a double-blind, randomized trial. Hepatology 1987, 7:1278-1284.
64. Muto Y., Sato S., Watanabe A., et al. Effects of oral branched-chain amino acid granules on event-free survival in patients with liver cirrhosis. Clin Gastroenterol Hepatol 2005, 3:705-713.
65. Norenberg M.D., Rama Rao K.V., Jayakumar A.R. Signaling factors in the mechanism of ammonia neurotoxicity. Metab Brain Dis 2009, 24:103-117.
66. Parsons-Smith B.G., Summerskill W.H.J., Dawson A.M., et al. The electroencephalograph in liver disease. Lancet 1957, ii:867-871.
67. Penin H. Über den diagnostischen Wert des Hirnstrombildes bei der hepato-portalen Encephalopathie. Fortschr Neurol Psychiatr 1967, 35:174-234.
68. Phillips G., Schwartz R., Gabuzda G., et al. The syndrome of impending hepatic coma in patients with cirrhosis of the liver given certain nitrogenous substances. N Engl J Med 1952, 247:239-246.
69. Plum F., Hindfelt B. The neurological complications of liver disease. Handbook of Clinical Neurology 1976, vol. 27:349-377. Elsevier, Amsterdam. P. Vinken, D.F. Bruyn (Eds.).
70. Pomier-Layrargues G., Rose C., Spahr L., et al. Role of manganese in the pathogenesis of portal-systemic encephalopathy. Metab Brain Dis 1998, 13:311-317.
71. Poo J.L., Gongora J., Sanchez-Avila F., et al. Efficacy of oral L-ornithine-L-aspartate in cirrhotic patients with hyperammonemic hepatic encephalopathy. Results of a randomized, lactulose-controlled study. Ann Hepatol 2006, 5:281-288.
72. Poveda M.-J., Bernabeu A., Concepción L., et al. Brain edema dynamics in patients with overt hepatic encephalopathy. A magnetic resonance imaging study. Neuroimage 2010, 52:481-487.
73. Prasad S., Dhiman R.K., Duseja A., et al. Lactulose improves cognitive functions and health-related quality of life in patients with cirrhosis who have minimal hepatic encephalopathy. Hepatology 2007, 45:549-559.
74. Pujol A., Pujol J., Graus F., et al. Hyperintense globus pallidus on T1-weighted MRI in cirrhotic patients is associated with severity of liver failure. Neurology 1993, 43:65-69.
75. Quero J.C., Hartmann I.J., Meulstee J., et al. The diagnosis of subclinical hepatic encephalopathy in patients with cirrhosis using neuropsychological tests and automated electroencephalogram analysis. Hepatology 1996, 24:556-560.
76. Randolph C. The Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) 1998, Psychological Corporation, San Antonio.
77. Randolph C., Hilsabeck R., Kato A., et al. Neuropsychological assessment of hepatic encephalopathy: ISHEN practice guidelines. Liver Int 2009, 29:629-635.
78. Read A., Sherlock S., Laidlaw J., et al. The neuro-psychiatric syndromes associated with chronic liver disease and an extensive portal-systemic collateral circulation. Q J Med 1967, 36:135-150.
79. Rehnström S., Simert G., Hansson J.A., et al. Chronic hepatic encephalopathy. A psychometric study. Scand J Gastroenterol 1977, 12:305-311.
80. Rikkers L., Jenko P., Rudman D., et al. Subclinical hepatic encephalopathy: detection, prevalence, and relationship to nitrogen metabolism. Gastroenterology 1978, 75:462-469.
81. Romero-Gomez M., Boza F., Garcia-Valdecasas M.S., et al. Subclinical hepatic encephalopathy predicts the development of overt hepatic encephalopathy. Am J Gastroenterol 2001, 96:2718-2723.
82. Romero Gomez M., Cordoba J., Jover R., et al. Normality tables in the Spanish population for psychometric tests used in the diagnosis of minimal hepatic encephalopathy. Med Clin (Barc) 2006, 127:246-249.
83. Romero Gomez M., Cordoba J., Jover R., et al. Value of the critical flicker frequency in patients with minimal hepatic encephalopathy. Hepatology 2007, 45:879-885.
84. Rose C., Butterworth R.F., Zayed J., et al. Manganese deposition in basal ganglia structures results from both portal-systemic shunting and liver dysfunction. Gastroenterology 1999, 117:640-644.
85. Rose C., Michalak A., Rao K.V., et al. L-ornithine-L-aspartate lowers plasma and cerebrospinal fluid ammonia and prevents brain edema in rats with acute liver failure. Hepatology 1999, 30:636-640.
86. Rovira A., Grive E., Pedraza S., et al. Magnetization transfer ratio values and proton MR spectroscopy of normal appearing cerebral white matter in patients with liver cirrhosis. AJNR 2001, 22:1137-1142.
87. Schliess F., Görg B., Häussinger D. Pathogenetic interplay between osmotic and oxidative stress: the hepatic encephalopathy paradigm. Biol Chem 2006, 387:1363-1370.
88. Schomerus H., Hamster W. Neuropsychological aspects of portal-systemic encephalopathy. Metab Brain Dis 1998, 13:361-377.
89. Schomerus H., Hamster W., Blunck H., et al. Latent portasystemic encephalopathy: I. Nature of cerebral functional defects and their effect on fitness to drive. Dig Dis Sci 1981, 26:622-630.
90. Schomerus H., Weissenborn K., Hamster W., et al. PSE-Syndrom-Test 1999, Swets Test Services. Swets and Zeitlinger B.V., Frankfurt.
91. Shah N.J., Neeb H., Kircheis G., et al. Quantitative cerebral water content mapping in hepatic encephalopathy. Neuroimage 2008, 41:706-717.
92. Sharma P., Sharma B.C., Puri V., et al. Critical flicker frequency: diagnostic tool for minimal hepatic encephalopathy. J Hepatol 2007, 47:67-73.
93. Sharma B.C., Sharma P., Agarwal A., et al. Secondary prophylaxis of hepatic encephalopathy: an open label randomized controlled trial of lactulose versus placebo. Gastroenterology 2009, 137:885-891.
94. Shawcross D.L., Balata S., Olde-Damink S.W., et al. Low myoinositol and high glutamine levels in brain are associated with neuropsychological deterioration after induced hyperammonemia. Am J Physiol 2004, 287:G503-G509.
95. Shawcross D.L., Davies N.A., Williams R., et al. Systemic inflammatory response exacerbates the neuropsychological effects of induced hyperammonemia in cirrhosis. J Hepatol 2004, 40:247-254.
96. Shawcross D.L., Olde Damink S.W.M., Butterworth R.F., et al. Ammonia and hepatic encephalopathy: the more things change, the more they remain the same. Metab Brain Dis 2005, 20:169-179.
97. Sherlock S. Pathogenesis and management of hepatic coma. Am J Med 1958, 24:805-813.
98. Sherlock S., Summerskill W.H.J., White L.P., et al. Portal-systemic encephalopathy. neurological complications of liver disease. Lancet 1954, I:453-457.
99. Singhal A., Nagarajan R., Kumar R., et al. Magnetic resonance T2-relaxometry and 2D L-correlated spectroscopy in patients with minimal hepatic encephalopathy. J Magn Reson Imaging 2009, 30:1034-1041.
100. Spahr L., Butterworth R.F., Fontaine S., et al. Increased blood manganese in cirrhotic patients: relationship to pallidal magnetic resonance signal hyperintensity and neurological symptoms. Hepatology 1996, 24:1116-1120.
101. Stauch S., Kircheis G., Adler G., et al. Oral L-ornithine-L-aspartate therapy of chronic hepatic encephalopathy: results of a placebo-controlled double-blind study. J Hepatol 1998, 28:856-864.
102. Summerskill W.H.J., Davidson E.A., Sherlock S., et al. The neuropsychiatric syndrome associated with hepatic cirrhosis and an extensive portal collateral circulation. Q J Med 1956, 25:245-266.
103. Tarter R.E., Hegedus A.M., Van Thiel D.H., et al. Nonalcoholic cirrhosis associated with neuropsychological dysfunction in the absence of overt evidence of hepatic encephalopathy. Gastroenterology 1984, 86:1421-1427.
104. Thumburu K.K., Kurmi R., Dhiman R.K., et al. Psychometric hepatic encephalopathy score, critical flicker frequency and P300 event-related potential for the diagnosis of minimal hepatic encephalopathy: evidence that psychometric hepatic encephalopathy score is enough. 13th ISHEN 2008, Padua, 2008 [abstract].
105. Uribe M., Campollo O., Vargas F., et al. Acidifying enemas (lactitol and lactose) vs. nonacidifying enemas (tap water) to treat acute portal-systemic encephalopathy: a double-blind randomized clinical trial. Hepatology 1987, 7:639-643.
106. Van der Rijt C.D.C., Schalm S., De Groot G.H., et al. Objective measurement of hepatic encephalopathy by means of automated EEG analysis. Electroencephalogr Clin Neurophysiol 1990, 75:289-295.
107. Victor M., Adams R.D., Cole M. The acquired (non-Wilsonian) type of chronic hepatocerebral degeneration. Medicine (Baltimore) 1965, 44:345-396.
108. Watanabe A., Tuchida T., Yata Y., et al. Evaluation of neuropsychological function in patients with liver cirrhosis with special reference to their driving ability. Metab Brain Dis 1995, 10:239-248.
109. Wein C., Koch H., Popp B., et al. Minimal hepatic encephalopathy impairs fitness to drive. Hepatology 2004, 39:739-745.
110. Weissenborn K., Scholz M., Hinrichs H., Wiltfang J., Schmidt F.W., Künkel H. Neurophysiological assessment of early hepatic encephalopathy. Electroencephalogr Clin Neurophysiol. 1990, 75(4):289-295.
111. Weissenborn K. Neurophysiological methods in the diagnosis of early hepatic encephalopathy. Progress in Hepatic Encephalopathy and Metabolic Nitrogen Exchange 1991, 27-39. CRC Press, Boca Raton. F. Bengtsson, B. Jeppsson, T. Almdal (Eds.).
112. Weissenborn K., Ennen J., Schomerus H., et al. Neuropsychological characterisation of hepatic encephalopathy. J Hepatol 2001, 34:768-773.
113. Weissenborn K., Tietge U.J., Bokemeyer M., et al. Liver transplantation improves hepatic myelopathy: evidence by three cases. Gastroenterology 2003, 124:346-351.
114. Wunsch E., Koziarska D., Kotarska K., et al. Normalization of the psychometric hepatic encephalopathy score in polish population. A prospective, quantified electroencephalography study. Liver Int 2013, 33:1332-1340.
115. Zeegen R., Drinkwater J.E., Dawson A.M. Method for measuring cerebral dysfunction in patients with liver disease. Br Med J 1970, 2:633-636.
116. Zieve L. Historical remarks and recent trends in hepatic encephalopathy. Progress in Hepatic Encephalopathy and Metabolic Nitrogen Exchange 1991, 3-8. CRC Press, Boca Raton. F. Bengtsson, B. Jeppsson, T. Almdal (Eds.).