Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

It is generally assumed that neuronal cell death is minimal in liver failure and is insufficient to account for the neuropsychiatric symptoms characteristic of hepatic encephalopathy. However, contrary to this assumption, neuronal cell damage and death are well documented in liver failure patients, taking the form of several distinct clinical entities namely acquired (non-Wilsonian) hepatocerebral degeneration, cirrhosis-related Parkinsonism, post-shunt myelopathy and cerebellar degeneration. In addition, there is evidence to suggest that liver failure contributes to the severity of neuronal loss in Wernicke's encephalopathy. The long-standing nature of the thalamic and cerebellar lesions, over 80% of which are missed by routine clinical evaluation, together with the probability that they are nutritional in origin, underscores the need for careful nutritional management (adequate dietary protein, Vitamin B(1)) in liver failure patients. Mechanisms identified with the potential to cause neuronal cell death in liver failure include NMDA receptor-mediated excitotoxicity, lactic acidosis, oxidative/nitrosative stress and the presence of pro-inflammatory cytokines. The extent of neuronal damage in liver failure may be attenuated by compensatory mechanisms that include down-regulation of NMDA receptors, hypothermia and the presence of neuroprotective steroids such as allopregnanolone. These findings suggest that some of the purported "sequelae" of liver transplantation (gait ataxia, memory loss, confusion) could reflect preexisting neuropathology.
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PMID:Neuronal cell death in hepatic encephalopathy. 1785 42

A 53-year-old woman visited us for a neurological consultation before her liver transplantation. She had a history of primary biliary cirrhosis that began at 37 years of age. She showed falling episodes and met with a traffic accident at 52 years old. Since then, her symptoms had worsened. The neurological examination showed masked face, rigidity on bilateral arms and legs, and lack of balance. Her Mini-Mental State Examination Score was 28/30, but she suffered from loss of memory and had trouble with executive function in detailed examinations. Her T(1) weighted image showed hyperintensity in bilateral globus pallidus, putamen, dentate nucleus and cerebral peduncle. There was a significant improvement in intellectual function and neurological signs 6 months after her orthotopic liver transplantation. In addition, post-liver transplantation images showed a decrease in the area of hyperintensities. This case suggests that even in a patient with severe liver cirrhosis a complete cure of neurological manifestations can be obtained after the liver transplantation.
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PMID:[Case of acquired hepatocerebral degeneration with prominent improvement of parkinsonism and cognitive deficits after living-donor liver transplantation]. 2297 58