Acquired hepatocerebral degeneration

Changed by Daniel J Bell, 29 Mar 2022

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Acquired hepatocerebral degeneration is an uncommon irreversible extrapyramidal neurodegenerative condition encountered in patients with cirrhotic chronic liver disease, resulting in widespread cerebral, basal ganglia, and cerebellar damage. 

Terminology

Acquired hepatocerebral degeneration is a term that is restricted to patients which cirrhotic liver disease resulting from a variety of causes but specifically excluding Wilson disease; in fact, this condition is often termed acquired non-Wilsonian hepatocerebral degeneration to ensure the distinction is made. 

Acute neurological dysfunction from hyperammonaemia or other transient alterations in hepatic metabolism is termed hepatic encephalopathy and is discussed separately. Occasionally acquired hepatocerebral degeneration is referred to as chronic hepatic encephalopathy, although this is probably best avoided. 

Clinical presentation

Patients with acquired hepatocerebral degeneration usually, but not always, have had multiple prior episodes of hepatic encephalopathy 6. They present with gradual neurological dysfunction including dementia, rigidity, dysarthria, gait ataxia, tremor, and choreoathetosis 5.

Pathology

Although the pathophysiology of acquired hepatocerebral degeneration is uncertain, manganesemanganese overload is believed to be part of the disease and responsible for the T1 shortening observed in the globus pallidus 7

Radiographic features

MRI
  • T1
    • intrinsic hyperintensity in the globus pallidus, subthalamic region, and midbrain (substantia nigra)1-5
    • thought to be a reflection of increased tissue concentrations of manganese 1-4
    • may reverse following liver transplantation 6
  • T2/FLAIR
    • increased signal in the middle cerebellar peduncles (MCP sign) 5

Treatment and prognosis

Acquired hepatocerebral degeneration is generally thought to be irreversible, although recovery following liver transplantation has been reported 6

Differential diagnosis

  • -<p><strong>Acquired hepatocerebral degeneration</strong> is an uncommon irreversible extrapyramidal neurodegenerative condition encountered in patients with <a href="/articles/cirrhosis">cirrhotic</a> chronic liver disease, resulting in widespread cerebral, basal ganglia, and cerebellar damage. </p><h4>Terminology</h4><p>Acquired hepatocerebral degeneration is a term that is restricted to patients which cirrhotic liver disease resulting from a variety of causes but specifically excluding <a href="/articles/wilson-disease-2">Wilson disease</a>; in fact, this condition is often termed <strong>acquired non-Wilsonian hepatocerebral degeneration</strong> to ensure the distinction is made. </p><p>Acute neurological dysfunction from hyperammonaemia or other transient alterations in hepatic metabolism is termed <a href="/articles/hepatic-encephalopathy">hepatic encephalopathy</a> and is discussed separately. Occasionally acquired hepatocerebral degeneration is referred to as chronic hepatic encephalopathy, although this is probably best avoided. </p><h4>Clinical presentation</h4><p>Patients with acquired hepatocerebral degeneration usually, but not always, have had multiple prior episodes of <a href="/articles/hepatic-encephalopathy">hepatic encephalopathy</a> <sup>6</sup>. They present with gradual neurological dysfunction including dementia, rigidity, dysarthria, gait ataxia, tremor, and choreoathetosis <sup>5</sup>.</p><h4>Pathology</h4><p>Although the pathophysiology of acquired hepatocerebral degeneration is uncertain, manganese overload is believed to be part of the disease and responsible for the T1 shortening observed in the globus pallidus <sup>7</sup>. </p><h4>Radiographic features</h4><h5>MRI</h5><ul>
  • +<p><strong>Acquired hepatocerebral degeneration</strong> is an uncommon irreversible extrapyramidal neurodegenerative condition encountered in patients with <a href="/articles/cirrhosis">cirrhotic</a> chronic liver disease, resulting in widespread cerebral, basal ganglia, and cerebellar damage. </p><h4>Terminology</h4><p>Acquired hepatocerebral degeneration is a term that is restricted to patients which cirrhotic liver disease resulting from a variety of causes but specifically excluding <a href="/articles/wilson-disease-2">Wilson disease</a>; in fact, this condition is often termed <strong>acquired non-Wilsonian hepatocerebral degeneration</strong> to ensure the distinction is made. </p><p>Acute neurological dysfunction from hyperammonaemia or other transient alterations in hepatic metabolism is termed <a href="/articles/hepatic-encephalopathy">hepatic encephalopathy</a> and is discussed separately. Occasionally acquired hepatocerebral degeneration is referred to as chronic hepatic encephalopathy, although this is probably best avoided. </p><h4>Clinical presentation</h4><p>Patients with acquired hepatocerebral degeneration usually, but not always, have had multiple prior episodes of <a href="/articles/hepatic-encephalopathy">hepatic encephalopathy</a> <sup>6</sup>. They present with gradual neurological dysfunction including dementia, rigidity, dysarthria, gait ataxia, tremor, and choreoathetosis <sup>5</sup>.</p><h4>Pathology</h4><p>Although the pathophysiology of acquired hepatocerebral degeneration is uncertain, <a title="Manganese" href="/articles/manganese">manganese</a> overload is believed to be part of the disease and responsible for the T1 shortening observed in the globus pallidus <sup>7</sup>. </p><h4>Radiographic features</h4><h5>MRI</h5><ul>

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