Acquired hepatocerebral degeneration

Introduction
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By Fermina Pirmohamed MD and Leslie J Cloud MD MSc

A minority of patients with chronic liver disease develop acquired hepatocerebral degeneration – a progressive neurologic disorder characterized by extrapyramidal signs, ataxia, and cognitive decline. Although the pathogenesis of acquired hepatocerebral degeneration is not known, diversion of portal blood into the systemic circulation appears to underlie the syndrome. In this clinical summary, Dr. Fermina Pirmohamed and Dr. Leslie Cloud of Virginia Commonwealth University review the clinical, radiological, and pathological features of acquired hepatocerebral degeneration along with current treatment strategies. Evidence supporting and opposing the potential role of manganese in acquired hepatocerebral degeneration’s pathogenesis is discussed, as well as outcomes following liver transplantation.

Key points

  • Acquired hepatocerebral degeneration is a neurologic disorder that is clinically distinct from hepatic encephalopathy and characterized by extrapyramidal signs, ataxia, and cognitive dysfunction.
  • The pathogenesis of acquired hepatocerebral degeneration is not known, but portosystemic shunting likely plays a central role.
  • Acquired hepatocerebral degeneration is not a contraindication to liver transplantation; indeed, in some cases transplantation may be the only effective therapy.

In This Article

Introduction
Historical note and nomenclature
Clinical manifestations
Etiology
Pathogenesis and pathophysiology
Epidemiology
Prevention
Differential diagnosis
Diagnostic workup
Prognosis and complications
Management
Pregnancy
Anesthesia
References cited
Contributors