Abstract：
:We discuss the case of a rare and often unrecognized neurologic syndrome, called Acquired Hepatocerebral Degeneration (AHD), observed in patients with advanced liver disease and portosystemic shunts. The clinical manifestations can be very heterogeneous and in our case included a combination of cerebellar and extrapyramidal signs, arisen in a period of few days. Brain Magnetic Resonance Imaging (MRI) showed, in T1-weighted images, diffuse bilateral hyper intensities in basal ganglia and biemispheric brain and cerebellar cortices, resembling paramagnetic deposits. No other neurological impairments, like stroke, infection or neoplasia, were found. It was excluded an episode of acute hepatic encephalopathy. We also ruled out Wilsonian degeneration, iron overload and autoimmune encephalitis and we lastly found high manganese levels as the possible cause of the brain paramagnetic deposits. Even though either serum Mn determination or its accumulation in the brain are not specific for AHD, however the chronic and progressively worsening of the neurological manifestations advocated a degenerative condition, possibly AHD. We finally opted for the early restoration of liver function by OLT, and we observed complete clinical symptoms' resolution and partial MRI reversal after a follow up of 6 months.
journal_name
Metab Brain Disjournal_title
Metabolic brain diseaseauthors
Pigoni A,Iuculano F,Saetti C,Airaghi L,Burdick L,Spreafico S,Curioni M,Lombardi R,Valenti L,Fracanzani AL,Fargion Sdoi
10.1007/s11011-017-0107-0subject
Has Abstractpub_date
2018-02-01 00:00:00pages
347-352issue
1eissn
0885-7490issn
1573-7365pii
10.1007/s11011-017-0107-0journal_volume
33pub_type
杂志文章abstract：:Studies have shown that oxidative stress is involved in the pathophysiology of bipolar disorder (BD). It is suggested that omega-3 (ω3) fatty acids are fundamental to maintaining the functional integrity of the central nervous system. The animal model used in this study displayed fenproporex-induced hyperactivity, a s...
journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-016-9942-7
更新日期：2017-04-01 00:00:00
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journal_title：Metabolic brain disease
pub_type： 杂志文章,评审
doi：10.1007/s11011-016-9906-y
更新日期：2016-12-01 00:00:00
abstract：:The availability of an animal model is crucial in studying the pathophysiological mechanisms of disease and to test possible therapies. Now, there are several models for the study of liver diseases, but there still remains a lack of a satisfactory animal model of chronic liver disease with hepatic encephalopathy (HE) ...
journal_title：Metabolic brain disease
pub_type： 杂志文章,评审
doi：10.1007/s11011-005-7925-1
更新日期：2005-12-01 00:00:00
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journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-012-9349-z
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journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1023/a:1020631029168
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journal_title：Metabolic brain disease
pub_type： 杂志文章
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journal_title：Metabolic brain disease
pub_type： 杂志文章
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journal_title：Metabolic brain disease
pub_type： 杂志文章,评审
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journal_title：Metabolic brain disease
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doi：10.1007/s11011-016-9880-4
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journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-014-9643-z
更新日期：2015-06-01 00:00:00
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journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-017-9988-1
更新日期：2017-08-01 00:00:00
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journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/BF02098880
更新日期：1994-12-01 00:00:00
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journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-008-9126-1
更新日期：2009-03-01 00:00:00
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journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-018-0309-0
更新日期：2018-12-01 00:00:00
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journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-020-00609-z
更新日期：2020-12-01 00:00:00
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journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-015-9673-1
更新日期：2015-10-01 00:00:00
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journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-006-9002-9
更新日期：2006-03-01 00:00:00
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journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-017-0180-4
更新日期：2018-06-01 00:00:00
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journal_title：Metabolic brain disease
pub_type： 杂志文章
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journal_title：Metabolic brain disease
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journal_title：Metabolic brain disease
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更新日期：2016-08-01 00:00:00
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journal_title：Metabolic brain disease
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更新日期：2015-02-01 00:00:00
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journal_title：Metabolic brain disease
pub_type： 杂志文章,评审
doi：10.1023/a:1021941414605
更新日期：2002-12-01 00:00:00
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journal_title：Metabolic brain disease
pub_type： 杂志文章
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journal_title：Metabolic brain disease
pub_type： 杂志文章
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更新日期：2017-10-01 00:00:00
abstract：:High plasma levels of methionine (Met) and its metabolites such as methionine sulfoxide (MetO) may occur in several genetic abnormalities. Patients with hypermethioninemia can present neurological dysfunction; however, the neurotoxicity mechanisms induced by these amino acids remain unknown. The aim of the present wor...
journal_title：Metabolic brain disease
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abstract：:Lactic acid accumulation has been implicated in the evolution of brain damage after ischemia. Since compartmentation of lactate may play a role in acid-base balance, lactate release from gerbil hippocampal slices was examined during a number of metabolic stresses including elevated [K+]e, ischemia, anoxia, and aglycem...
journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/BF00999841
更新日期：1990-09-01 00:00:00
abstract：:Sleep disturbances are prevalent among patients with Alzheimer's disease (AD) and often precede the onset and progression of dementia. However, there are no reliable animal models for investigating sleep disturbances in patients with sporadic AD (sAD), which accounts for more than 90% of all AD cases. In the present s...
journal_title：Metabolic brain disease
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doi：10.1007/s11011-017-0125-y
更新日期：2018-02-01 00:00:00
