Acquired hepatocerebral degeneration in a liver transplant recipient

Soffer, Dov; Sherman, Yoav; Tur–Kaspa, Ran; Eid, Ahmed

doi:10.1007/s004010050306

Cited by 4 publications

(7 citation statements)

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“…Characteristically, polymicrocavitation extends into adjacent white matter tracts where it appears to displace rather than destroy surrounding neurons. In some cases, however, white matter vacuolization is accompanied by myelinolysis [66,78,137]. Polymicrocavitation does not involve the cerebellum, and ataxia in patients with AHD may be related to degeneration of the dentate nuclei, Purkinje cell loss, and Bergmann's gliosis.…”

Section: Pathogenesismentioning

confidence: 94%

“…Within the cortex, polymicrocavitation is often most severe in the posterior frontal, parietal and occipital areas [30], but the temporal lobes may also be affected [66,137]. In some patients, polymicrocavitation is greatest adjacent to sulci [148,154], while in others, it is more prominent at the gyral summits [30, 66,137,144]. Within the basal ganglia, the superior pole of the putamen, caudate heads, pallidum and subthalamic nuclei are most involved [36, 66,154].…”

Section: Pathogenesismentioning

confidence: 99%

“…Vacuolar change originates in the deep cortical layers and basal ganglia. Within the cortex, polymicrocavitation is often most severe in the posterior frontal, parietal and occipital areas [30], but the temporal lobes may also be affected [66,137]. In some patients, polymicrocavitation is greatest adjacent to sulci [148,154], while in others, it is more prominent at the gyral summits [30, 66,137,144].…”

Section: Pathogenesismentioning

confidence: 99%

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Acquired hepatocerebral degeneration

2009

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Cirrhosis and its co-morbidities may cause a variety of neurological complications, the most common being bouts of toxic metabolic encephalopathy. A proportion of patients with chronic liver disease develop acquired hepatocerebral degeneration (AHD), a chronic progressive neurological syndrome characterized by parkinsonism, ataxia and other movement disorders. This article reviews the clinical spectrum, pathophysiology, neuroimaging features and differential diagnosis of AHD along with emerging treatment options.

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Section: Pathogenesismentioning

confidence: 94%

Section: Pathogenesismentioning

confidence: 99%

Section: Pathogenesismentioning

confidence: 99%

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Acquired hepatocerebral degeneration

2009

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“…Adapted from Bechar et al (1970) following either multiple episodes of severe HE (coma) or prolonged time in coma, and is characterized by neurodegenerative changes in cerebral cortex, basal ganglia and cerebellum (Fig. 3) (Butterworth 2007;Soffer et al 1995). The coexistence of neurodegenerative changes and Alzheimer type II astrocytosis in thalamus and cerebellum of HE patients has been noted (Kril and Butterworth 1997).…”

Section: Acquired Non-wilsonian Hepatocerebral Degenerationmentioning

confidence: 95%

“…This complication occurs most frequently in patients Soffer et al (1995). b Cerebellar degeneration in a cirrhotic patient showing co-existence of Purkinje cell loss and Alzheimer type II astrocytosis (arrow).…”

Section: Acquired Non-wilsonian Hepatocerebral Degenerationmentioning

confidence: 97%

Metal Toxicity, Liver Disease and Neurodegeneration

Butterworth

2010

Neurotox Res

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Hepatocerebral disorders are serious neuropsychiatric conditions that result from liver failure. These disorders are characterized neuropathologically by varying degrees of neuronal cell death in basal ganglia, cerebellum, and spinal cord, and include clinical entities such as Wilson's Disease, post-shunt myelopathy, hepatic encephalopathy, and acquired non-Wilsonian hepatocerebral degeneration. Morphologic changes to astrocytes (Alzheimer type II astrocytosis) are a major feature of hepatocerebral disorders. Neurological symptoms include Parkinsonism, cognitive dysfunction, and ataxia. Pathophysiologic mechanisms responsible for cerebral dysfunction and neuronal cell death in hepatocerebral disorders include ammonia toxicity and neurotoxic effects of metals such as copper, manganese, and iron. Molecular mechanisms of neurotoxicity include oxidative/nitrosative stress, glutamate (NMDA)-receptor-mediated excitotoxicity, and neuroinflammatory mechanisms. However, neuronal cell death in hepatocerebral disorders is limited by adaptive mechanisms that may include NMDA-receptor down-regulation, the synthesis of neuroprotective steroids and hypothermia. Management and treatment of hepatocerebral disorders include chelation therapy (Wilson's Disease), the use of ammonia-lowering agents (lactulose, antibiotics, ornithine aspartate) and liver transplantation.

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