The Acquired (Non-Wilsonian) Type of Chronic Hepatocerebral Degeneration

Improvement in chronic hepatocerebral degeneration following liver transplantation

“…The neurological symptoms and signs manifested by this patient comprise the syndrome of the acquired type of chronic hepatocerebral degeneration as described by Victor et al (2). Earlier reports by Parkes et al (7) and Chu et al (8) have described electroencephalographic and somatosensory-evoked potential changes following OLT.…”

“…Acute HE is reversed by dietary protein restriction, lactulose, and neomycin, and is worsened by hypokalemia, constipation, and drugs that increase γ-aminobutyric acid-ergic activity (9,10). However, the chronic neuropsychiatric syndromes and chronic hepatocerebral degeneration have been regarded as largely irreversible and caused mostly by organic structural changes in the brain and spinal cord (2). It is noteworthy that significant improvement in the neuropsychiatric syndrome has been defined following prolonged lactulose usage and by Freund et al (11) using a branched chain-enriched amino acid diet.…”

“…Acute HE is a potentially reversible encephalopathy due to a metabolic disturbance, probably causing widespread alteration in cerebral neurotransmission (1). This should be distinguished from the less-common chronic progressive encephalopathies such as "acquired hepatocerebral degeneration" (2) and the persistent neuropsychiatric syndromes associated with cirrhosis (3). Chronic progressive encephalopathy is characterized by dementia, dysarthria, ataxia, tremor, extrapyramidal signs, and spastic paraparesis (2,4).…”

“…This should be distinguished from the less-common chronic progressive encephalopathies such as "acquired hepatocerebral degeneration" (2) and the persistent neuropsychiatric syndromes associated with cirrhosis (3). Chronic progressive encephalopathy is characterized by dementia, dysarthria, ataxia, tremor, extrapyramidal signs, and spastic paraparesis (2,4). It is more common in patients with large portal-systemic shunts, and usually does not respond to therapy for HE.…”

“…It is more common in patients with large portal-systemic shunts, and usually does not respond to therapy for HE. Structural changes in the central nervous system have been reported in association with this rare, more persistent neuropsychiatric syndrome (2,3). Liver transplantation provides an opportunity to study the extent to which the chronic neuropsychiatric syndrome is reversible.…”