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Metabolische Enzephalopathien

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Klinische Neurologie

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Zusammenfassung

Metabolische Enzephalopathien stellen oft eine diagnostische Herausforderung dar, da sie sich klinisch sehr variabel präsentieren, und – außer im Falle einer schweren Hypo- oder Hyperglykämie – regelhaft andere mögliche Ursachen der neurologischen Symptomatik ausgeschlossen werden müssen, bevor die Diagnose »metabolische Enzephalopathie« gestellt werden kann. Gerade in Zeiten zunehmender Spezialisierung schärfen sie den Blick für das Ganze und erinnern daran, dass der Mensch mehr ist als die Summe seiner Organe. Sie zeigen auch, dass gute Medizin eine gute Kommunikation zwischen den Disziplinen voraussetzt.

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Literatur

Zitierte Literatur

  • Amodio P, Gatta A (2005) Neurophysiological investigation of hepatic encephalopathy. Metabol Brain Dis 20(4): 369–379

    Article  Google Scholar 

  • Andermann E, Andermann F, Carpenter S, Wolfe LS, Nelson R, Patry G, Boileau J (1986) Action myoclonus-renal failure syndrome: A previously unrecognized neurological disorder unmasked by advances in nephrology. Adv Neurol 43: 87–103

    PubMed  CAS  Google Scholar 

  • Atterbury CE, Maddrey W, Conn HO (1978) Neomycin-sorbitol and lactulose in the treatment of acute portal-systemic encephalopathy. A controlled, double-blind clinical trial. Am J Dig Dis 23: 398–406

    Article  PubMed  CAS  Google Scholar 

  • Bajaj JS, Hafeezullah M, Hoffmann RG et al. (2008) Navigation skill impairment: Another dimension of the driving difficulties in minimal hepatic encephalopathy. Hepatology 47(2): 596–604

    Article  PubMed  Google Scholar 

  • Bhatia V, Batra Y, Acharya, SK (2004) Prophylactic phenytoin does not improve cerebral edema or survival in acute liver failure – a controlled clinical trial. J Hepatology 41: 89–96

    Article  CAS  Google Scholar 

  • Bhatia V, Singh R, Acharya SK (2006) Predictive value of arterial ammonia for complications and outcome in acute liver failure. GUT 55(1): 98–104

    Article  PubMed  CAS  Google Scholar 

  • Bergui M, Bradac GB, Zhong JJ, Barbero PA, Durelli L (2001) Diffusion-weighted MR in reversible Wernicke encephalopathy. Neuroradiology 43: 969–972

    Article  PubMed  CAS  Google Scholar 

  • Brouns R, De Deyn PP (2004) Neurological complications in renal failure: A review. Clin Neurol Neurosurg 107: 1–16

    Article  PubMed  CAS  Google Scholar 

  • Bustamante J, Rimola A, Ventura PJ, Navasa M, Cirera I, Reggiardo V, Rodes J (1999) Prognostic significance of hepatic encephalopathy in patients with cirrhosis. J Hepatol 30: 890–895

    Article  PubMed  CAS  Google Scholar 

  • Cadranel J-F, Lebiez E, Di Martino V (2001) Focal neurological signs in hepatic encephalopathy in cirrhotic patients: An underestimated entity? Am J Gastroenterol 96(2): 515–518

    Article  PubMed  CAS  Google Scholar 

  • Chu K, Kang DW, Kim HJ, Lee YS, Park SH (2002) Diffusion-weighted imaging abnormalities in Wernicke encephalopathy: Reversible cytotoxic edema? Arch Neurol 59(1): 123–127

    Article  PubMed  Google Scholar 

  • Cordoba J, Lopez-Hellin J, Planas M et al. (2004) Normal protein diet for episodic hepatic encephalopathy: Results of a randomized study. J Hepatol 41: 38–43

    Article  PubMed  CAS  Google Scholar 

  • Davies MG, Rowan MJ, Feely J. (1991) EEG and event-related potentials in hepatic encephalopathy. Metab Brain Dis 6(4): 175–186

    Article  PubMed  CAS  Google Scholar 

  • Diabetes Control and Complications Trial Research Group (1997) Hypoglycemia in the Diabetes Control and Complications Trial. Diabetes 46(2): 271–286

    Article  Google Scholar 

  • Elias MF, Elias PK, Seliger SL, Narsipur SS, Dore GA, Robbins MA (2009) Chronic kidney disease, creatinine and cognitive functioning. Nephrol Dial Transplant 24(8): 2446–2452

    Article  PubMed  CAS  Google Scholar 

  • Ellis AJ, Wendon JA, Williams R (2000) Subclinical seizure activity and prophylactic phenytoin infusion in acute liver failure: A controlled clinical trial. Hepatology 32: 536–541

    Article  PubMed  CAS  Google Scholar 

  • Evers St, Tepel M, Obladen M, Suhr B, Husstedt I-W, Grotemeyer KH, Zidek W (1998) Influence of end stage renal failure and hemodialysis on eventrelated potentials. J Clin Neurophysiol 15(1): 58–63

    Article  PubMed  CAS  Google Scholar 

  • Ferenci P, Lockwood AH, Mullen K, Tarter R, Weissenborn K, Blei AT (2002) Hepatic encephalopathy – definition, nomenclature, diagnosis, and quantification: Final report of the working party at the 11th World Congress of Gastroenterology, Vienna 1998. Hepatology 35(3) 716–721

    Article  PubMed  Google Scholar 

  • Ferrara J, Jankovic J (2009) Acquired hepatocerebral degeneration. J Neurology 256(3): 320–332

    Article  Google Scholar 

  • Hartmann IJC, Groeneweg M, Quero JC et al. (2000) The prognostic significance of subclinical hepatic encephalopathy. Am J Gastroenterol 95: 2029–2034

    Article  PubMed  CAS  Google Scholar 

  • Hui AY, Chan HL, Leung NW, Hung LC, Chan FK, Sung JJ (2002) Survival and prognostic indicators in patients with hepatitis B virus-related cirrhosis after onset of hepatic decompensation. J Clin Gastroenterol 34(5): 569–572

    Article  PubMed  Google Scholar 

  • Ihara M, Ito T, Yanagihara C, Nishimura Y (1999) Wernicke’s encephalopathy associated with hemodialysis: Report of two cases and review of the literature. Clin Neurol Neurosurg 101: 118–121

    Article  PubMed  CAS  Google Scholar 

  • Jalan R, Olde Damink SW, Deutz NE, Hayes PC, Lee A (2004) Moderate hypothermia in patients with acute liver failure and uncontrolled intracranial hypertension. Gastroenterology 127: 1338–1346

    Article  PubMed  CAS  Google Scholar 

  • Jones EA, Weissenborn K (1997) Neurology and the Liver. J Neurol Neurosurg Psychiatry 63(3): 279–293

    Article  PubMed  CAS  Google Scholar 

  • Kandiah N, Tan K, Lim CCT, Venketasubramanian N (2009) Hyperglycemic choreoathetosis. Movement Disorders 24(6): 915–936

    Article  PubMed  Google Scholar 

  • Kearney T, Dang C (2007) Diabetic and endocrine emergencies. Postgrad Med J 83: 79–86

    Article  PubMed  CAS  Google Scholar 

  • Kircheis G, Nilius R, Held C et al. (1997) Therapeutic efficacy of L-ornithine-Laspartate infusions in patients with cirrhosis and hepatic encephalopathy: Results of a placebo-controlled, double-blind study. Hepatology 25: 1351–1360

    Article  PubMed  CAS  Google Scholar 

  • Kircheis G, Wettstein M, Timmermann L, Schnitzler A, Haussinger D (2002) Critical flicker frequency for quantification of low-grade hepatic encephalopathy. Hepatology 35: 357–366

    Article  PubMed  Google Scholar 

  • Kircheis G, Knoche A, Hilger N, Manhart F, Schnitzler A, Schulze H, Häussinger D (2009) Hepatic encephalopathy and fitness to drive. Gastroenterology 137(5): 1706–1715

    Article  PubMed  Google Scholar 

  • Kitabchi AE, Umpierrez GE, Murphy MB et al. (2004) Hyperglycemic crises in diabetes. Diabetes Care 27(suppl 1): S94–S102

    PubMed  Google Scholar 

  • Kitabchi AE, Umpierrez GE, Miles JM, Fisher JN (2009) Hyperglycemic crises in adult patients with diabetes. Diabetes Care 32(7): 1335–1343

    Article  PubMed  CAS  Google Scholar 

  • Kodl CT, Seaquist ER (2008) Cognitive dysfunction and diabetes mellitus. Endocr Rev 29(4): 494–511

    Article  PubMed  CAS  Google Scholar 

  • Krieger S, Jauß M, Jansen O, Theilmann L, Geißler M, Krieger D (1996) Neuropsychiatric profile and hyperintense lobus pallidus on T1-weighted magnetic resonance images in liver cirrhosis. Gastroenterology 111: 147–155

    Article  PubMed  CAS  Google Scholar 

  • Kril JJ, Butterworth RF (1997) Diencephalic and cerebellar pathology in alcoholic and nonalcoholic patients with end-stage liver disease. Hepatology 26: 837–841

    Article  PubMed  CAS  Google Scholar 

  • Leevy CB, Phillips JA (2007) Hospitalizations during the use of rifaximin versus lactulose for the treatment of hepatic encephalopathy. Dig Dis Sci 52: 737–741

    Article  PubMed  CAS  Google Scholar 

  • Levin DL (2008) Cerebral edema in diabetic ketoacidosis. Pediatr Crit Care Med 9: 320–329

    Article  PubMed  Google Scholar 

  • Lockwood AH (1989) Neurological complications of renal disease. Neurologic Clinics 7(3): 617–627

    PubMed  CAS  Google Scholar 

  • Lockwood AH (2000) »What’s in a name?« Improving the care of cirrhotics. J Hepatol 32: 859–861

    Article  PubMed  CAS  Google Scholar 

  • Mahoney CA, Arieff AI (1982) Uremic encephalopathies: Clinical, biochemical, and experimental features. AmJ Kidney Dis 2(3): 324–336

    CAS  Google Scholar 

  • Marchesini G, Bianchi G, Merli M et al. (2003) Nutritional supplementation with branched-chain amino acids in advanced cirrhosis: A double-blind, randomized trial. Gastroenterology 124: 1792–1801

    Article  PubMed  CAS  Google Scholar 

  • Mas A, Rodes J, Sunyer L et al.; Spanish Association for the study of the Liver Hepatic Encephalopathy Cooperative Group (2003) Comparison of rifaximin and lactitol in the treatment of acute hepatic encephalopathy: Results of a randomized, double-blind, double-dummy, controlled clinical trial. J Hepatol 38: 51–58

    Article  PubMed  CAS  Google Scholar 

  • Moe SM, Sprague SM (1994) Uremic encephalopathy. Clin Nephrology 42(4): 251–256

    CAS  Google Scholar 

  • Morgan MY (1998) Cerebral magnetic resonance imaging in patients with chronic liver disease. Metab Brain Dis 13(4): 273–290

    Article  PubMed  CAS  Google Scholar 

  • Munoz SJ (1993) Difficult management problems in fulminant hepatic failure. Semin Liver Disease 13: 395–413

    Article  CAS  Google Scholar 

  • Murphy N, Auzinger G, Bernal W, Wendon J (2004) The effect of hypertonic sodium chloride on intracranial pressure in patients with acute liver failure. Hepatology 39: 464–470

    Article  PubMed  CAS  Google Scholar 

  • Muto Y, Sato S, Watanabe A et al. for the Long-Term-Survival Study (LOTUS) Group (2005) Effects of oral branched-chain amino acid granules on event-free survival in patients with liver cirrhosis. Clin Gastroenterol Hepatol 3: 705–713

    Article  PubMed  CAS  Google Scholar 

  • O’Grady JG, Schalm SW, Williams R (1993) Acute liver failure: Redefining the syndromes. Lancet 342: 273–275

    Article  PubMed  Google Scholar 

  • Ostapowicz GA, Fontana RJ, Schiodt FV et al. (2002) Results of a prospective study of acute liver failure at 17 tertiary care centers in the United States. Ann Intern Med 137: 947–954

    PubMed  Google Scholar 

  • Penin H (1967) Über den diagnostischen Wert des Hirnstrombildes bei der hepato-portalen Encephalopathie. Fortschr Neurol Psychiatr Grenzgeb 35: 174–234

    PubMed  CAS  Google Scholar 

  • Plum F, Posner JB (1982) The diagnosis of stupor and coma, 3rd edn. Davis, Philadelphia, pp 1–86

    Google Scholar 

  • Polson J, Lee WM (2005) AASLD position paper: The management of acute liver failure. Hepatology 41(5): 1179–1197

    Article  PubMed  Google Scholar 

  • Poo JL, Gongora J, Sanchez-Avila F et al. (2006) Efficacy of oral L-ornithine-Laspartate in cirrhotic patients with hyperammonemic hepatic encephalopathy. Results of a randomized, lactulose-controlled study. Ann Hepatol 5(4): 281–288

    PubMed  Google Scholar 

  • Powell EE, Pender MP, Chalk JB et al. (1990) Improvement in chronic hepatocerebral degeneration following liver transplantation. Gastroenterology 98: 1079–1082

    Article  PubMed  CAS  Google Scholar 

  • Prasad S, Dhiman RK, Duseja A, Chawla YK, Sharma A, Agarwal R (2007) Lactulose improves cognitive functions and health-related quality of life in patients with cirrhosis who have minimal hepatic encephalopathy. Hepatology 45: 549–559

    Article  PubMed  Google Scholar 

  • Quadri AM, Ogunwale BO, Mullen KD (2007) Can we ignore minimal hepatic encephalopathy any longer? Hepatology 45: 547–548

    Article  Google Scholar 

  • Randolph C, Hilsabeck R, Kato A et al. (2009) International Society for Hepatic Encephalopathy and Nitrogen Metabolism (ISHEN). Neuropsychological assessment of hepatic encephalopathy: ISHEN practice guidelines. Liver Int 29(5): 629–635

    Article  PubMed  Google Scholar 

  • Rinella ME, Sanyal A (2006) Intensive management of hepatic failure. Sem Respiratory Crit Care Med 27(3): 241–261

    Article  Google Scholar 

  • Rose C, Butterworth RF, Zayed J et al. (1999) Manganese deposition in basal ganglia structures results from both portal-systemic shunting and liver dysfunction. Gastroenterology 117(3): 640–644

    Article  PubMed  CAS  Google Scholar 

  • Rovira A, Alonso J, Córdoba J (2008) MR imaging findings in hepatic encephalopathy. Am J Neuroradiol 29(9): 1612–1621

    Article  PubMed  CAS  Google Scholar 

  • Russo G, Conte P, Sallusto L, Di Maggio A, Scatizzi A (1989) Electrophysiologic follow-up (brainstem acoustic evoked potentials and visual evoked potentials) in chronic uremic patients treated with various therapeutic protocols. Riv Neurol 59(6): 229–238

    PubMed  CAS  Google Scholar 

  • Sass DA, Shakil AO (2005) Fulminant hepatic failure. Liver Transplantat 11 (6): 594–605

    Article  Google Scholar 

  • Savage MW, Mah PM, Weetman AP, Newell-Price J (2004) Endocrine emergencies. Postgrad Med 80: 506–515

    Article  CAS  Google Scholar 

  • Schomerus H, Hamster W (2001) Quality of life in cirrhotics with minimal hepatic encephalopathy. Metab Brain Dis 16: 13–19

    Article  Google Scholar 

  • Schomerus H, Schreiegg J (1993) Prevalence of latent portasystemic encephalopathy in an unselected population of patients with liver cirrhosis in general practice. Z Gastroenterol 31: 231–234

    PubMed  CAS  Google Scholar 

  • Schomerus H, Hamster W, Blunck H, Reinhard K, Mayer K, Dölle W (1981) Latent portasystemic encephalopathy: I. Nature of cerebral functional defects and their effect on fitness to drive. Dig Dis Sci 26: 622–630

    Article  PubMed  CAS  Google Scholar 

  • Shami VM, Caldwell SH, Hespenheide EE, Arseneau KO, Blickston SJ, Macik BG (2003) Recombinant activated factor VII for coagulopathy in fulminant hepatic failure compared with conventional therapy. Liver Transpl 9: 138–143

    Article  PubMed  Google Scholar 

  • Souheaver GT, Ryan JR, Dewolfe AS (1982) Neuropsychological patterns in uremia. J Clin Psychol 38(3): 490–496

    Article  PubMed  CAS  Google Scholar 

  • Stoner GD (2005) Hyperosmolar hyperglycemic state. Am Fam Physician 71: 1723–1730

    PubMed  Google Scholar 

  • Spahr L, Butterworth RF, Fontaine S et al. (1996) Increased blood manganese in cirrhotic patients: Relationship to pallidal magnetic resonance signal hyperintensity and neurological symptoms. Hepatology 24: 1116–1120

    Article  PubMed  CAS  Google Scholar 

  • Srivastava A, Mehta R, Rothke SP, Rademaker AW, Blei AT (1994) Fitness to drive in patients with cirrhosis and portal systemic shunting: A pilot study evaluating driving performace. J Hepatol 21: 1023–1028

    Article  PubMed  CAS  Google Scholar 

  • Tofteng F, Larsen FS (2004) The effect of indomethacin on intracranial pressure, cerebral perfusion and extracellular lactate and glutamate concentrations in patients with fulminant hepatic failure. J Cereb Blood Flow Metab 24: 79–804

    Google Scholar 

  • Vaquero J, Fontana RJ, Larson AM (2005) Complications and use of intracranial pressure monitoring in patients with acute liver failure and severe encephalopathy. Liver Transpl 12: 1581–1589

    Article  Google Scholar 

  • Victor M, Adams RD, Cole M (1965) The acquired (non-Wilsonina) type of chronic hepatocerebral degeneration. Medicine (Baltimore) 44: 345–394

    Article  CAS  Google Scholar 

  • Wang HC, Brown P, Lees AJ (1998) Acute movement disorders with bilateral basal ganglia lesions in uremia. Mov Disord 13(6): 952–957

    Article  PubMed  CAS  Google Scholar 

  • Watanabe A, Tuchida T, Yata Y, Kuwabara J (1995) Evaluation of neuro-psychological function in patients with liver cirrhosis with special reference to their driving ability. Metab Brain Dis 10: 239–248

    Article  PubMed  CAS  Google Scholar 

  • Wein C, Koch H, Popp B, Oehler G, Schauder P (2004) Minimal hepatic encephalopathy impairs fitness to drive. Hepatology 39(3): 739–745

    Article  PubMed  Google Scholar 

  • Weissenborn K, Scholz M, Hinrichs H, Wiltfang J, Schmidt FW, Künkel H (1990) Neurophysiological assessment of early hepatic encephalopathy. Electroencephalogr Clin Neurophysiol 75: 289–295

    Article  PubMed  CAS  Google Scholar 

  • Weissenborn K, Ennen JC, Schomerus H, Ruckert N, Hecker H (2001) Neuropsychological characterization of hepatic encephalopathy. J Hepatol 34: 768–773

    Article  PubMed  CAS  Google Scholar 

  • Weissenborn K, Tietge UJ, Bokemeyer M, Mohammadi B, Bode U, Manns MP, Caselitz M (2003) Liver transplantation improves hepatic myelopathy: evidence by three cases. Gastroenterology 124: 346–351

    Article  PubMed  Google Scholar 

  • Windebank AJ, Feldman EL (2001) Diabetes and the nervous system. In: Aminoff MJ (Hrsg) Neurology and general medicine. Churchill Livingstone, Philadelphia, pp 341–364

    Google Scholar 

  • Wolfsdorf J, Glaser N, Sperling MA (2006) Diabetic ketoacidosis in infants, children, and adolescents. A consensus statement from the American Diabetes Association. Diabetes Care 29: 1150–1159

    Article  PubMed  Google Scholar 

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  1. Neurologische Klinik, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover

    Prof. Dr. Karin Weissenborn

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  1. Klinik für Neurologie, Alfried Krupp Krankenhaus Rüttenscheid, Alfried-Krupp-Straße 21, 45117, Essen

    Peter Berlit

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Weissenborn, K. (2011). Metabolische Enzephalopathien. In: Berlit, P. (eds) Klinische Neurologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-16920-5_36

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