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         Hepatocerebral Encephalopathy:     more detail

41. Marchiafava-Bignami Syndrome - Patient UK
Other brain lesions associated with alcoholism, eg Wernicke s encephalopathy,hepatocerebral degeneration, head trauma, central pontine myelinolysis and
http://www.patient.co.uk/showdoc/40001259/
PatientPlus articles are written for doctors and so the language can be technical. However, some people find that they add depth to the articles found in the other sections of this website which are written for non-medical people.
Marchiafava-Bignami Syndrome
First described by Marchiafava and Bignami (2 Italian pathologists) in 1903 . A progressive neurological disease most frequently seen in middle-aged or elderly alcoholic males. Degeneration, usually uniform, of the middle portion (middle lamina) of the myelinated fiber tracts of the corpus callosum occurs Epidemiology
  • About 150 cases have been reported.
  • Many cases may go undetected, with clinical features merging with other underlying alcohol-related problems
Risk Factors Most cases have been reported in:
  • Aged over 45
  • Male
  • Alcoholic
Presentation Symptoms
  • Most patients have a history of alcoholism and poor nutrition
  • Onset may be sudden with stupor, coma or seizures
  • Other patients present with acute or chronic dementia and/or gait problems. Spasticity often complicates the gait disorder.
  • Psychiatric disturbances include incontinence, hemiparesis, aphasia, and apraxia.

42. OTHER DISEASES OF DIGESTIVE SYSTEM
572.2 Hepatic coma. Hepatic encephalopathy hepatocerebral intoxication Portalsystemicencephalopathy. 572.3 Portal hypertension. 572.4 Hepatorenal syndrome
http://www.thera.info/icd9-cm/sect-570-579.html
OTHER DISEASES OF DIGESTIVE SYSTEM (570-579)
Acute and subacute necrosis of liver
Chronic liver disease and cirrhosis

Liver abscess and sequelae of chronic liver disease

Other disorders of liver
...
Intestinal malabsorption
570 Acute and subacute necrosis of liver
Acute hepatic failure
Acute or subacute hepatitis, not specified as infective
Necrosis of liver (acute) (diffuse) (massive) (subacute)
Parenchymatous degeneration of liver
Yellow atrophy (liver) (acute) (subacute)
Excludes: icterus gravis of newborn (773.0-773.2)
serum hepatitis (070.2-070.3) that with: abortion (634-638 with .7, 639.8) ectopic or molar pregnancy (639.8) pregnancy, childbirth, or the puerperium (646.7) viral hepatitis (070.0-070.9)
571 Chronic liver disease and cirrhosis
571.0 Alcoholic fatty liver
571.1 Acute alcoholic hepatitis
Acute alcoholic liver disease
571.2 Alcoholic cirrhosis of liver
Florid cirrhosis Laennec's cirrhosis (alcoholic)
571.3 Alcoholic liver damage, unspecified
571.4 Chronic hepatitis
Excludes: viral hepatitis (acute) (chronic) (070.0-070.9) 571.40 Chronic hepatitis, unspecified

43. Scope Of Work
Hepatic stupor and coma (hepatic or portalsystemic encephalopathy) Chronic acquired (Non-Wilsonian) hepatocerebral degeneration Kernicterus
http://www.cochraneneuronet.org/livello2/scope_of_work2.html
Index of Diseases
BACK to scope of work
ACQUIRED METABOLIC DISORDERS MIGRAINE AND HEADACHE ALCOHOL AND ALCOHOLISM ... OTHERS ACQUIRED METABOLIC DISORDERS back to index Ischemic-Hypoxic encephalopathy
Carbon Monoxide Poisoning
High-Altitude sickness
Hypercapnic pulmonary disease
Hypoglycemic encephalopathy
Hyperglycemia
Hepatic stupor and coma (hepatic or portal-systemic encephalopathy)
Uremic encephalopathy
Encephalopathy associated with sepsis and burns
Disorders of sodium, potassium and water balance Central pontine myelinolysis Chronic acquired (Non-Wilsonian) hepatocerebral degeneration Kernicterus Hypoparathyroidism Cerebellar ataxia associated with myxedema Effects of Hyperthermia on the Cerebellum Cerebellar syndromes associated with celiac-sprue and Jejunoileal bypass Cushing disease and corticosteroid psychoses Thyroid encephalopathies Pancreatic encephalopathy

44. ICD-9-CM From Code 570
Hepatic encephalopathy; hepatocerebral intoxication; Portalsystemic encephalopathy.572.3 Portal hypertension; 572.4 Hepatorenal syndrome
http://www.dmi.columbia.edu/hripcsak/icd9/1tabular570.html
OTHER DISEASES OF DIGESTIVE SYSTEM (570-579)
  • 570 Acute and subacute necrosis of liver
    • Acute hepatic failure
    • Acute or subacute hepatitis, not specified as infective
    • Necrosis of liver (acute) (diffuse) (massive) (subacute)
    • Parenchymatous degeneration of liver
    • Yellow atrophy (liver) (acute) (subacute)
    • Excludes: icterus gravis of newborn (773.0-773.2)
      • serum hepatitis (070.2-070.3)
      • that with:
        • abortion (634-638 with .7, 639.8)
        • ectopic or molar pregnancy (639.8)
        • pregnancy, childbirth, or the puerperium (646.7)
      • viral hepatitis (070.0-070.9)
    • 571 Chronic liver disease and cirrhosis
      • 571.0 Alcoholic fatty liver
      • 571.1 Acute alcoholic hepatitis
        • Acute alcoholic liver disease
      • 571.2 Alcoholic cirrhosis of liver
        • Florid cirrhosis
        • Laennec's cirrhosis (alcoholic)
      • 571.3 Alcoholic liver damage, unspecified
      • 571.4 Chronic hepatitis
        • Excludes: viral hepatitis (acute) (chronic) (070.0-070.9)
        • 571.40 Chronic hepatitis, unspecified
        • 571.41 Chronic persistent hepatitis
        • 571.49 Other
          • Chronic hepatitis:
            • active
            • aggressive
          • Recurrent hepatitis
        • 571.5 Cirrhosis of liver without mention of alcohol

45. Clinical Geriatrics
Psychosis in dementia may occur in alcoholic dementia, or dementia associatedwith hepatic encephalopathy (chronic hepatocerebral degeneration) and
http://www.hmpcommunications.com/cg/displayArticle.cfm?articleID=cgac1984

46. Free Online ICD9/ICD9CM Codes And Medical Dictionary
Hepatic encephalopathy. hepatocerebral intoxication. Portalsystemic encephalopathy.572.3 Portal hypertension. 572.4 Hepatorenal syndrome. Excludes
http://icd9cm.chrisendres.com/index.php?action=child&recordid=5128

47. Portal Toolkit Invalid Site URL
We report two patients with chronic acquired hepatocerebral degeneration (CAHD) who Hepatic encephalopathy is well known as one of the central nervous
http://ppv.ovid.com/pt/re/obes/fulltext.00000132-200208000-00007.htm
Sorry, the URL specified, http://ppv.ovid.com:80/pt/re/obes/fulltext.00000132-200208000-00007.htm , is invalid.

Thank you

48. Current Opinion In Neurology - UserLogin
Early detection and treatment of hepatic encephalopathy as more severe formsof encephalopathy or chronic nonWilsonian hepatocerebral degeneration.
http://www.co-neurology.com/pt/re/coneuro/fulltext.00019052-199812000-00009.htm
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49. ISNO Dutch Neuromuscular Research Support Centre - NMD
with distinct hepatocerebral and myopathic forms of this disorder. Mitochondrial encephalopathy lactic acidosis and strokelike episodes (MELAS)
http://www.isno.nl/sorteer/ziekten/?id=107

50. The Neurology Of Liver Failure -- Lewis And Howdle 96 (9): 623 -- QJM
In fulminant hepatic failure where hepatic encephalopathy develops within 8 The pathogenesis of both acquired hepatocerebral degeneration and hepatic
http://qjmed.oxfordjournals.org/cgi/content/full/96/9/623
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Request Permissions PubMed PubMed Citation Articles by Lewis, M. Articles by Howdle, P.D. Q J Med 2003;
Association of Physicians
Review
The neurology of liver failure
M. Lewis and P.D. Howdle
Introduction Top
Introduction
Overt hepatic encephalopathy
Minimal hepatic encephalopathy
Hepatic encephalopathy and liver... Fulminant hepatic failure Acquired hepatocerebral... Osmotic demyelination disorders Other neurological disorders in... Neuroimaging abnormalities... Conclusions References That there is a relationship between the brain and the liver has been known for many years

51. The Neurology Of Liver Failure -- Lewis And Howdle 96 (9): 623 -- QJM
Hepatic encephalopathy and liver transplantation. Fulminant hepatic failure.Acquired hepatocerebral degeneration and hepatic myelopathy
http://qjmed.oxfordjournals.org/cgi/content/extract/96/9/623
JOURNAL HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS QUICK SEARCH: [advanced] Author:
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This Article Full Text FREE Full Text (PDF) Alert me when this article is cited ... Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed ... Download to citation manager Search for citing articles in:
ISI Web of Science (1)
Request Permissions PubMed PubMed Citation Articles by Lewis, M. Articles by Howdle, P.D. Q J Med 2003;
Review
The neurology of liver failure
M. Lewis and P.D. Howdle The first 150 words of the full text of this article appear below.
Introduction
That there is a relationship between the brain and the liver has been known for many years, and patients with chronic liver disease frequently experience neurological problems. The commonest and most widely recognized is the reversible syndrome of hepatic encephalopathy, and we will deal with this in some detail.

52. Journal Of Computer Assisted Tomography - UserLogin
Acquired hepatocerebral degeneration is a well known irreversible neurologic Portalsystemic encephalopathy presence of basal ganglia lesions with high
http://www.jcat.org/pt/re/jcat/fulltext.00004728-199811000-00024.htm
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53. Journal Of Computer Assisted Tomography - UserLogin
Portosystemic encephalopathy was previously only thought to occur in adults with The hepatocerebral degeneration is attributed to the toxic effects of
http://www.jcat.org/pt/re/jcat/fulltext.00004728-200209000-00003.htm
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54. Transplantation - Fulltext: Volume 62(11) December 15, 1996 P 1679-1681 COMPLETE
Type II citrullinemia is an adultonset hepatocerebral disease caused by adeficiency of A 25-year-old Japanese man suddenly developed encephalopathy,
http://www.transplantjournal.com/pt/re/transplantation/fulltext.00007890-1996121
LWWOnline LOGIN eALERTS REGISTER ... Archive COMPLETE NEUROLOGICAL RECOVERY... ARTICLE LINKS:
Abstract
References (14) View full size inline images Transplantation Volume 62(11) 15 December 1996 pp 1679-1681
COMPLETE NEUROLOGICAL RECOVERY OF AN ADULT PATIENT WITH TYPE II CITRULLINEMIA AFTER LIVING RELATED PARTIAL LIVER TRANSPLANTATION
Yazaki, Masahide ; Ikeda, Shu-ichi ; Takei, Yo-ichi ; Yanagisawa, Nobuo ; Matsunami, Hidetoshi ; Hashikura, Yasuhiko ; Kawasaki, Seiji ; Makuuchi, Masatoshi ; Kobayashi, Keiko ; Saheki, Takeyori Third Department of Internal Medicine and First Department of Surgery, Shinshu University School of Medicine, Matsumoto 390; Second Department of Surgery, Faculty of Medicine, Tokyo University, Tokyo 114; and Department of Biochemistry, Faculty of Medicine, Kagoshima University, Kagoshima 890, Japan Third Department of Internal Medicine, Shinshu University School of Medicine. Address correspondence to: Shu-ichi Ikeda, MD, Third Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto 390, Japan. First Department of Surgery, Shinshu University School of Medicine.

55. Untitled Document
Tubulopathy, encephalopathy, and liver failure due to CIII deficiency no ortholog, Mitochondrial DNAdepletion syndrome, hepatocerebral form (MDS)
http://www-deletion.stanford.edu/YDPM/doc/mtdisease_yeasthomolog.html
Web Table B. Yeast orthologs to known human mitochondrial disease genes
gene name protein ID yeast ortholog homozygous deletion disease class III Wilson disease (WD) BCKDHA DEHUXA class III Maple syrup urine disease (MSUD) BCKDHB class III Maple syrup urine disease (MSUD) class III Tubulopathy, encephalopathy, and liver failure due to CIII deficiency class III Deficiency of complex IV DBT class III Maple syrup urine disease (MSUD) DLD DEHULP class III Dihydrolipoamide dehydrogenase deficiency;Leigh syndrome FH UFHUM class III Deficiency of fumarate hydratase GCSH GCHUH class III Non-ketotic hyperglycinemia, type III (NKH3) HHH class III Deficiency of ornithine translocase class III Deficiency of MTHFD1 DEHUPA class III Pyruvate dehydrogenase deficiency;Leigh syndrome class III Pyruvate dehydrogenase deficiency POLG class III Progressive external ophthalmoplegia with mitochondrial DNA deletions (PEO); Involved in male infertility (MI)

56. Cirrhosis
Is associated with mental confusion, ie encephalopathy. part of the spectrumof acquired hepatocerebral degeneration). Grading of hepatic encephalopathy
http://janis7hepc.com/cirrhosis3.htm
Home Cirrhosis Back to Index 2005 Research 2004-2001 Research Archives PHYSICAL FINDINGS SUGGESTIVE OF CIRRHOSIS A liver biopsy is the only definitive test that actually indicates whether or not you have cirrhosis [irreversible scaring of the liver]. So, what exactly is your doctor looking for when he/she does a physical exam? Are there actually some physical clues that suggest that you have cirrhosis? The answer to this question is yes. The following is a list of clues that indicate that you may already have cirrhosis. These are listed alphabetically by their common medical name. It is important to remember that, although helpful, each of these physical manifestations do not indicate the actual cause of one's liver disease, and, in fact, are not specific only to liver disease. They can be due to other disorders as well. ASCITES An accumulation of excess fluid in the abdomen. Causes abdominal distention. Can be treated with a low sodium diet, and the use of diuretics, i.e. water pills. Ascites:
The two most important factors in the development of ascites are failure of the liver to synthesize albumin and portal venous hypertension.

57. Articles
Do Ammonia Levels Correlate with Hepatic encephalopathy? of the spectrum ofacquired hepatocerebral degeneration). Grading of hepatic encephalopathy
http://janis7hepc.com/articles1.htm
Home Back to Index Medical Problems and Cirrhosis Antibiotic prophylaxis after variceal hemorrhage reduces incidence of early rebleeding Normal protein diet for episodic hepatic encephalopathy Grading of hepatic encephalopathy Screening for Varices in Patients With Cirrhosis: ... Do Ammonia Levels Correlate with Hepatic Encephalopathy? Hepatogastroenterology. 2004 Mar-Apr;51(56):541-6.
Antibiotic prophylaxis after variceal hemorrhage reduces incidence of early
rebleeding.
Pohl J, Pollmann K, Sauer P, Ring A, Stremmel W, Schlenker T.
Department of Internal Medicine IV, Ruprechts-Karls-University, Heidelberg,
Germany. juergen_pohl@med.uni-heidelberg.de
BACKGROUND/AIMS: This study aims to evaluate the role of new onset infection
in the initiation of early rebleeding after variceal hemorrhage in patients
with liver cirrhosis and the effect of prophylactic antibiotic treatment.
METHODOLOGY: Two hundred and twenty-one consecutive admissions for variceal
bleeding with no signs of infection at the time of admission were evaluated retrospectively. RESULTS: Systemic antibiotic prophylaxis was administered

58. *601465 DEOXYGUANOSINE KINASE; DGUOK
Affected individuals with the hepatocerebral form of mtDNA depletion syndrome hepatomegaly, hypoglycemia, jaundice, and encephalopathy with hypotonia,
http://srs.sanger.ac.uk/srsbin/cgi-bin/wgetz?[omim-ID:601465] -e

59. 231670 GLUTARIC ACIDEMIA I
cardiomyopathy, and Reyelike hepatocerebral crisis, and the latter can occurduring intercurrent Infantile encephalopathy; Spastic diplegia;
http://srs.sanger.ac.uk/srsbin/cgi-bin/wgetz?[omim-ID:231670] -e

60. Magnetic Resonance Imaging And Spectroscopy In Hepatic Encephalopathy. Gupta RK,
Hepatic encephalopathy (HE) is a potentially reversible neuropsychiatric In conclusion, MRI and MRS examine different aspects of hepatocerebral disease.
http://www.indianjgastro.com/article.asp?issn=0254-8860;year=2003;volume=22;issu

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