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Related Concept Videos

Hepatic Encephalopathy01:29

Hepatic Encephalopathy

DefinitionHepatic encephalopathy is a reversible neurologic syndrome that results from advanced liver dysfunction or portosystemic shunting. It leads to disturbances in cognition, behavior, and motor function due to the brain’s exposure to gut-derived toxins that the liver fails to detoxify.EtiologyThis condition develops either in the setting of acute fulminant hepatitis or progressively during chronic liver disease, such as cirrhosis and portal hypertension. Portosystemic shunting—including...
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Hepatitis is an inflammatory condition of the liver most commonly caused by hepatotropic viruses (A–E), though non-infectious causes such as alcohol and drugs also exist.Hepatitis AHepatitis A virus (HAV) is a non-enveloped RNA virus of the Picornaviridae family. It is primarily transmitted via the fecal-oral route, typically through ingestion of contaminated food or water. After ingestion, HAV enters the bloodstream through the oropharynx or intestinal epithelium and reaches the liver. The...
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Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...
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Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...
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Dementia is an acquired, progressive syndrome characterized by a decline in multiple cognitive domains severe enough to impair daily functioning and reduce independence. Although memory loss is a central feature, the diagnosis requires additional deficits involving language, executive function, visuospatial skills, judgment, calculation, or abstract reasoning. These cognitive impairments reflect underlying neurodegenerative or vascular processes that gradually disrupt neuronal networks...
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Cirrhosis is a progressive chronic liver injury caused by prolonged inflammation, excessive fibrotic remodeling, and impaired regeneration. Over time, repeated hepatic insults disrupt the liver’s architecture and function, leading to reduced blood flow, impaired bile drainage, and diminished metabolic capacity.Pathophysiology of cirrhosisCirrhosis arises from three main responses to chronic liver damage: inflammation, immune activation, and hepatocyte death. These processes lead to structural...

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Symmetric Bihemispheric Postmortem Brain Cutting to Study Healthy and Pathological Brain Conditions in Humans
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Published on: December 18, 2016

Acquired hepatocerebral degeneration.

Pushpendra Nath Renjen1, Laxmi Khanna, Ruchi Rastogi

  • 1Department of Neurology, Indraprastha Apollo Hospital, New Delhi, India. pnrenjen@hotmail.com

BMJ Case Reports
|June 20, 2013
PubMed
Summary

Acquired hepatocerebral degeneration, a rare neurological disorder linked to chronic liver disease, presents with cognitive decline and motor symptoms. This case highlights a patient with a portal systemic shunt experiencing these neurodegenerative effects.

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Area of Science:

  • Neurology
  • Hepatology
  • Neurodegenerative Diseases

Background:

  • Acquired hepatocerebral degeneration is a rare, irreversible neurological syndrome associated with chronic liver disease and metabolic insults.
  • First described in 1914, it shares clinical and pathological similarities with Wilson's disease.

Observation:

  • The primary risk factor identified is multiple episodes of hepatic coma, suggesting brain toxicity.
  • The condition affects 0.8-2% of patients with cirrhosis.

Findings:

  • This report details a case in a 50-year-old male with a long-standing portal systemic shunt.
  • The patient presented with progressive cognitive decline, bradykinesia, tremors, and bilateral extrapyramidal signs.

Implications:

  • This case underscores the importance of recognizing acquired hepatocerebral degeneration in patients with chronic liver disease and shunts.
  • Further research into subtle diagnostic markers, like glial fibrillar acidic protein immunostaining differences, is warranted.