Hepatic encephalopathy

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Classification according to ICD-10
B15.0 Viral hepatitis A with coma hepaticum
B16.0 Acute viral hepatitis B with Delta virus (accompanying infection) and with coma hepaticum
B16.2 Acute viral hepatitis B without delta virus with coma hepaticum
B19.0 Unspecified viral hepatitis with coma
K70.4 Alcoholic liver failure (with or without coma hepaticum)
K72 Liver failure, not elsewhere classified
K72.0 Acute and subacute liver failure
K72.1 Chronic liver failure
K72.7 Hepatic encephalopathy and coma hepaticum (grading is based on the West Haven criteria.)
K72.71 Grade 1 hepatic encephalopathy
K72.72 Grade 2 hepatic encephalopathy
K72.73 Grade 3 hepatic encephalopathy
K72.74 Grade 4 hepatic encephalopathy
K72.9 Liver failure, unspecified
ICD-10 online (WHO version 2019)

The hepatic encephalopathy (HE, synonyms: portosystemic encephalopathy , formerly portocaval encephalopathy ), brain liver disorder also called, is a potentially reversible disorder of the brain caused by insufficient detoxification function of the liver arises as a result of most chronic liver disease. The course of the disease leads to an increasing deterioration in cognitive and motor skills and can lead to so-called hepatic coma ( coma hepaticum , hepatic coma).

Prevalence and mortality

Around one million people in Germany suffer from cirrhosis of the liver ; 80% of the inpatients treated develop a hepatic encephalopathy, of which 36% of the patients develop a manifest and around half a minimal hepatic encephalopathy. As one of the main complications of alcohol-induced liver cirrhosis, it has the highest mortality rate compared to the other complications. According to a study, 45% of untreated patients who had hepatic encephalopathy in addition to other complications died within one month of the diagnosis; after one year it was 64%, after five years 85%. Because of this, it is necessary that a liver-brain disorder be diagnosed early.

Pathogenesis

The impaired detoxification function of the liver is the result of acute liver failure or chronic liver disease (e.g. cirrhosis of the liver with the formation of portocaval bypass circuits ), but also of a therapeutically applied shunt , which leads to an increase in the concentration of various substances in the body. Mention should be made here:

Ammonia is considered to be one of the major players in the pathophysiology of hepatic encephalopathy. Due to the insufficient metabolism by the liver, the blood in the intestinal tract is enriched with ammonia, from where it then enters the body's bloodstream. Ammonia enters the brain via the blood-brain barrier and is absorbed by the astrocytes . The astrocytes metabolize the absorbed ammonia to glutamine, which has an osmotic effect on the astrocyte volume. The resulting swelling of the astrocytes, which can also contribute to the formation of brain edema , is considered to be a major cause of hepatic encephalopathy.

Forms, degrees of severity and symptoms

A distinction is made between two forms of the course of the disease, persistent and episodic hepatic encephalopathy.

  • Persistent HE: This form is characterized by a permanent impairment of consciousness and continuous impairment of the patient's cognitive functions.
  • Episodic HE: In this course of the disease, symptom-free phases alternate with sections of neurological disturbances of consciousness (tiredness, decreased performance). However, the symptoms do not completely subside; rather, the neuro-cognitive disorders often increase with each subsequent HE episode.

West Haven classification: HE is divided into five degrees of severity according to the West Haven classification.

  • Minimal HE:
    The minimal HE is the least pronounced form of hepatic encephalopathy. In this form, no clinical symptoms are recognizable, but the patients show the first mild cognitive deficits that can adversely affect quality of life (HRQoL). In particular, complex activities that require attention, information processing and psychomotor skills are restricted. This includes, for example, driving a motor vehicle. Patients with MHE have higher rates of traffic violations and traffic accidents. Validated psychometric tests can provide an indication of minimal HE.
  • Subclinical HE:
    • Stage I:
      Slight reduction in the level of consciousness with increasing need for sleep, mood swings, irritability, clear drive disorder and decrease in intellectual performance. Noticeable disorders of fine motor skills with a change in the typeface, beginning tremors and slowed movement.
  • Manifestos HE:
    • Stage II:
      Considerable reduction in the level of consciousness with disorientation, pronounced memory impairment, impoverishment of emotional life and delayed response to speech. Slurred speech ( dysarthria ), "flapping tremor" and increased muscle tension.
    • III. Stage:
      Severe disturbance of consciousness ( Sopor : mostly sleeping, but awakenable patient), loss of orientation, confusion, incoherent speech, reduced reaction to painful stimuli. Increased muscle tension up to muscle stiffness ( spasticity ), stool and urinary incontinence, unsteadiness to walk and stand ( ataxia ). Severe sleepiness ( somnolence :)
    • IV. Stage:
      unconsciousness without reaction to painful stimuli ( coma ). Extinction of the muscular reflexes, muscle stiffness with flexion and extension posture, in the advanced stage loss of muscle tension.

The minimal HE and stage I of the West Haven classification are also summarized under the term Covert HE, while Stages II-IV are also referred to as Overt HE.

diagnosis

Validated psychometric tests, as recommended according to the current guidelines of the EASL and AASLD, can provide an indication of the minimal hepatic encephalopathy. These include so-called paper-pencil tests, such as the number connection test or line tracking test, which determine the psychometric hepatic encephalopathy score (PHES).

The manifest form of hepatic encephalopathy is diagnosed primarily on the basis of the clinical picture. Clinical and laboratory tests such as laboratory tests (e.g. liver function test, blood glucose, ammonia level), imaging methods (e.g. MRI, skull CT) and electrophysiological diagnostics (e.g. EEG) are used. This is primarily intended to exclude other causes for the neurological and metabolic disorders.

A high ammonia level in the blood - in patients with chronic liver disease - alone has only little diagnostic value. It does not yet allow an assignment to a HE degree. Likewise, no prognosis can be made on the basis of blood ammonia levels. The diagnosis “HE” should also be checked in the case of blood ammonia levels in the normal range.

therapy

Medication

  • Lactulose is a synthetic type of sugar ( disaccharide ) made from galactose and fructose and influences the intestinal flora in the sense of an excess weight of lactic acid- producing intestinal bacteria, which suppresses ammonia-producing intestinal bacteria and inhibits their urease , which catalyzes ammonia production. Furthermore, at the now lower pH value, ammonia is protonated to ammonium, which is excreted as a salt.
  • Neomycin was a local antibiotic used in the pastwhich, when administered orally , becomes effective in the intestine and kills the ammonia-producing bacteria there. However, due to the ototoxic and nephrotoxic side effects, treatment with neomycin is no longer recommended.
  • Rifaximin , a practically non-absorbable and locally acting antibiotic. In individual cases, combination therapy with rifaximin can be considered in acute therapy. As a monotherapy, it is used in hepatic encephalopathy grade 1 or higher (West Haven classification) only in the case of intolerance to lactulose.
  • L-ornithine-L-aspartate (LOLA) is a salt consisting of the two amino acids ornithine and aspartate. L-ornithine-L-aspartate (LOLA) iv can support ammonia detoxification in two ways: It activates both the impaired formation of urea in the liver and glutamine synthesis in the liver, muscles and brain.

Since hepatic encephalopathy tends to recur and the cognitive damage increases with each episode, consistent and long-term prophylaxis by the family doctor is very important. This also corresponds to the current guideline of the EASL and AASLD, which recommend long-term secondary prophylaxis in hepatic encephalopathy.

literature

  • S2k guideline for complications of liver cirrhosis of the German Society for Gastroenterology, Digestive and Metabolic Diseases eV (DGVS). In: AWMF online (as of 2018)
  • Hepatic encephalopathy in chronic liver disease: 2014 practice guideline by the European Association for the Study of the Liver and the American Association for the Study of Liver Diseases . In: J Hepatol , 2014, 61, pp. 642-659, doi: 10.1016 / j.jhep.2014.05.042 .
  • Christian Born: Hepatic Encephalopathy. A neuropsychological follow-up examination in 25 patients before and after liver transplantation. Dissertation . Humboldt University Berlin , 1997/1999.
  • Peter Ferenci, Karin Weissenborn: Hepatic Encephalopathy. UNI-MED Verlag, Bremen et al. 2002, ISBN 3-89599-622-X .
  • Dieter Häussinger, Klaus-Peter Maier (ed.): Hepatic encephalopathy. Thieme, Stuttgart et al. 1996, ISBN 3-13-104051-3 .
  • Erwin Kuntz (Ed.): The hepatic encephalopathy. Aspects of diagnosis and treatment; 3rd ornithine aspartate workshop, Gravenbruch; June 22, 1991. University of Jena , 1992, ISBN 3-86007-040-1 .
  • Hans-Christian Hansen (Ed.): Disturbances of consciousness and encephalopathies: diagnosis, therapy, prognosis. Springer-Verlag, Berlin / Heidelberg, ISBN 978-3-642-36914-8 .

Individual evidence

  1. ↑ Hans Adolf Kühn: Diseases of the liver. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition ibid. 1961, pp. 847-875, here: p. 853 ( Coma hepaticum ).
  2. ↑ a b c d JS Bajaj, CM Schubert, DM Heuman, JB Wade, DP Gibson, A. Topaz, K. Saeian, M. Hafeezullah, DE Bell, RK Sterling, RT Stravitz, V. Luketic, MB White, AJ Sanyal : Persistence of cognitive impairment after resolution of overt hepatic encephalopathy . In: Gastroenterology , 2010, 138, pp. 2332-2340, doi: 10.1053 / j.gastro.2010.02.015
  3. ↑ C Labenz et al. In: Z Gastroenterol , 2017, 55 (8), pp. 741-747.
  4. ↑ P. Jepsen, P. Ott, PK Andersen, HT Sørensen, H. Vilstrup: Clinical course of alcoholic liver cirrhosis: a Danish population-based cohort study . In: Hepatology , 2010; 51 (5), pp. 1675-1682 doi: 10.1002 / hep.23500
  5. ↑ Hans-Christian Hansen (Ed.): Disturbances of consciousness and encephalopathies: diagnosis, therapy, prognosis. Springer-Verlag, Berlin / Heidelberg, ISBN 978-3-642-36914-8 .
  6. ↑ L. Friedman, E. Keeffe: Handbook of liver disease . 2011, Chapter 13: Hepatic encephalopathy , pp. 183-192.
  7. ^ H. Vilstrup et al .: Hepatic Encephalopathy in Chronic Liver Disease . In: J Hepatol , 2014, 61 (3), pp. 642-659, doi: 10.1016 / j.jhep.2014.05.042
  8. ^ T. Zhan, W. Stremmel: The diagnosis and treatment of minimal hepatic encephalopathy . In: Dtsch Ärztebl. Int , 2012; 109 (10), pp. 180–187 doi: 10.3238 / arztebl.2012.0180 .
  9. ↑ S. Prasad, RK Dhiman, A. Duseja, YK Chawla, A. Sharma, R. Agarwal: Lactulose improves cognitive functions and health-related quality of life in patients with cirrhosis who have minimal hepatic encephalopathy . In: Hepatology , 2007, 45, pp. 549-559.
  10. ↑ M. Groeneweg, JC Quero, I. De Bruijn et al .: Subclinical hepatic encephalopathy impairs daily functioning . In: Hepatology , 1998, 28, pp. 45-49.
  11. ^ H Schomerus, W. Hamster: Quality of life in cirrhotics with minimal hepatic encephalopathy . In: Metab. Brain. Dis. , 2001, 16, pp. 37-41.
  12. ^ RK Dhiman, YK Chawla: Minimal hepatic encephalopathy . In: Indian J Gastroenterol. , 2009 Jan-Feb; 28 (1), pp. 5-16, doi: 10.1007 / s12664-009-0003-6
  13. ↑ Hepatic encephalopathy in chronic liver disease: 2014 practice guideline by the European Association for the Study of the Liver and the American Association for the Study of Liver Diseases . In: J Hepatol , 2014, 61, pp. 642-659, doi: 10.1016 / j.jhep.2014.05.042
  14. ↑ Hans-Christian Hansen (Ed.): Disturbances of consciousness and encephalopathies: diagnosis, therapy, prognosis. Springer-Verlag, Berlin / Heidelberg, ISBN 978-3-642-36914-8 .
  15. ↑ H. Vilstrup, P. Amodio, J. Bajaj, J. Cordoba, P. Ferenci, KD Mullen et al .: Hepatic encephalopathy in chronic liver disease: 2014 Practice Guideline by the American Association for the Study of Liver Diseases and the European Association for the Study of the Liver . In: Hepatology , 2014, 61, pp. 642-659.
  16. ^ SM Riordan, R. Williams: Treatment of hepatic encephalopathy . In: The New England Journal of Medicine , 1997, vol. 337, pp. 473-479.
  17. ↑ S2k- guideline complications of cirrhosis of the German Society of Gastroenterology, Digestive and Metabolic Diseases Association (DGVS). In: AWMF online (as of 2018)
  18. ↑ C. Labenz et al. In: Z Gastroenterol , 2017, 55 (8), pp. 741-747.
  19. ^ T. Zhan, W. Stremmel: The diagnosis and treatment of minimal hepatic encephalopathy . In: Deutsches Ärzteblatt International , 2012, 109 (10), pp. 180–187, doi: 10.3238 / arztebl.2012.0180 .
  20. ↑ Hepatic encephalopathy in chronic liver disease: 2014 practice guideline by the European Association for the Study of the Liver and the American Association for the Study of Liver Diseases . In: J Hepatol , 2014, 61, pp. 642-659, doi: 10.1016 / j.jhep.2014.05.042 .