Acquired hepatocerebral degeneration: a case report

Abstract

Acquired hepatocerebral degeneration is an underdiagnosed neurologic syndrome characterized by parkinsonism, ataxia or other movement disorders and by neuropsychiatric and cognitive symptoms. It occurs in patients with chronic liver disease, especially those who develop portosystemic shunting and is often unrecognized as a cause of cognitive decline. Recently, its pathogenesis has been associated with manganese accumulation in basal ganglia and some treatments proposed. The aim of this article was to report a case and discuss some discoveries in connection with the disease.

Cognitive impairment and magnetic resonance imaging correlations in Wilson's disease

“…The presence of subclinical hepatic encephalopathy may lead to executive dysfunction , but only four patients from our sample had a prior history of liver dysfunction and, of these, two had normal cognitive performance, thus making this possibility unlikely. Acquired hepatolenticular degeneration can lead to motor and cognitive impairments , mimicking the findings of the present study. However, differently to what was found in our patients, in this condition, prominent visuospatial impairment, in addition to typical MRI changes (hypersignal in basal ganglia on T1), is evident, which was not observed in any of our cases.…”

Acquired hepatocerebral degeneration in a patient with hepatitis B and hepatitis delta virus coinfection

“…The pathogenesis of ADH has not yet been clearly elucidated. One hypothesis suggests that the presence of portosystemic shunts is a pre-condition to developing ADH 3,4,5 . This supposition is made because deviation of portal blood to the systemic circulation would result in the passage of neurotoxic substances, such as amino acids, ammonia, and heavy metals including manganese, that have not been removed by the hepatobiliary system, through the blood brain barrier into the central nervous system (CNS) [8][9][10] .…”

“…These changes exert a selective neurotoxic effect, affecting the integrity of neurons and astrocytes, mainly in the basal nuclei, brainstem, and white matter 8 . In the basal ganglia, manganese might accumulate in the mitochondria of the globus pallidus, thereby damaging the glial cells, as suggested by the diffuse hyperplasia of astrocytes, microcavitation, and zonal necrosis observed 5,6,9 . Moreover, this would also cause cellular loss and gliosis in the caudate nucleus, putamen, and subthalamus nucleus.…”

“…It usually presents with extrapyramidal signs (ataxia, Parkinsonism, chorea, athetosis, ballism, and cerebellar signs) and varying degrees of neuropsychiatric and cognitive impairments 4 . It has been described in patients with cirrhosis due to HBV infection, hepatitis C virus (HCV) infection, or alcoholism [3][4][5] . However, there are no case reports relating HBV/ HBD coinfection with AHD.…”

Acute stroke-like presentation of acquired hepatocerebral degeneration

“…Most cases of AHCD are diagnosed in patients with overt liver disease. [ 3 4 ] Review of literature describes AHCD as a chronic process occurring in patients with documented liver disease who have had repeated episodes of hepatic encephalopathy. In a case report by Noone et al ., two patients who presented with slowly progressive Parkinsonism were subsequently diagnosed to have cirrhosis based on subtle abnormalities in liver function tests and MRI findings.…”