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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...
Hepatitis01:25

Hepatitis

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...
Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

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,...
Encephalitis ll: Pathophysiology01:26

Encephalitis ll: Pathophysiology

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...
Dementia l: Introduction01:22

Dementia l: Introduction

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...
Cirrhosis II: Pathophysiology01:24

Cirrhosis II: Pathophysiology

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
08:29

Symmetric Bihemispheric Postmortem Brain Cutting to Study Healthy and Pathological Brain Conditions in Humans

Published on: December 18, 2016

Acquired hepatocerebral degeneration.

Wassilios Meissner1, François Tison

  • 1Department of Neurology, University Hospital of Bordeaux, University of Bordeaux 2, Bordeaux, France. wassilios.meissner@chu-bordeaux.fr

Handbook of Clinical Neurology
|April 19, 2011
PubMed
Summary

Acquired hepatocerebral degeneration (AHCD) results from liver issues, causing neurological symptoms. Manganese accumulation in the brain, particularly the basal ganglia, is implicated in AHCD pathogenesis.

Area of Science:

  • Neurology
  • Hepatology
  • Radiology

Background:

  • Acquired (non-Wilsonian) hepatocerebral degeneration (AHCD) is a neurological condition linked to liver failure or cirrhosis.
  • Patients may exhibit cognitive deficits, ataxia, dysarthria, movement disorders (including parkinsonism), and myelopathy.
  • Portosystemic shunting, often without abnormal liver function, is common in AHCD patients.

Purpose of the Study:

  • To elucidate the role of manganese in the pathogenesis of AHCD.
  • To describe the characteristic neuroimaging findings in AHCD.
  • To review potential therapeutic strategies for AHCD.

Main Methods:

  • Review of existing literature on AHCD, manganese metabolism, and neuroimaging.
  • Analysis of clinical presentations and diagnostic findings in AHCD patients.

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  • Evaluation of reported treatment outcomes for movement disorders associated with AHCD.

    • Main Results:

    • Manganese accumulation in the brain, especially the internal pallidum, is a key factor in AHCD, bypassing liver clearance due to portosystemic shunting.
    • MRI typically shows T1-weighted hyperintensity in the internal pallidum, putamen, caudate nucleus, and other brain regions, reflecting manganese deposition.
    • No definitive treatment exists, but case reports suggest potential benefits from branched-chain amino acid therapy, trientine, liver transplantation, and levodopa for parkinsonism.

    Conclusions:

    • Manganese neurotoxicity is central to AHCD development in patients with liver disease and portosystemic shunting.
    • Characteristic MRI findings aid in diagnosing AHCD by visualizing manganese accumulation.
    • Further controlled studies are needed to establish effective treatments for AHCD, though some interventions show promise.