Acquired hepatocerebral degeneration

Yalcin, Destina; Oguz‐Akarsu, Emel; Sokmen, Mehmet

doi:10.17712/nsj.2016.2.20150164

Cited by 8 publications

(3 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…It is characterised by manifestations such as parkinsonism, cognitive decline and movement disorders, forming a distinct profile of neurological complications [ 1 ] . The diagnosis of AHD relies on specific clinical criteria, including the presence of neurological manifestations, hyperintensity of the globus pallidus observed on T1-weighted brain MRI and the concurrent occurrence of CLD [ 4 ] .…”

Section: Discussionmentioning

confidence: 99%

Navigating diagnostic challenges: a case of acquired hepatocerebral degeneration presenting as worsening hepatic encephalopathy in chronic liver disease

Raza,

Bzowej,

Rini

et al. 2024

European Journal of Case Reports in Internal Medicine

View full text Add to dashboard Cite

This case report details the complex diagnostic odyssey of a 60-year-old female grappling with chronic liver disease, initially diagnosed with hepatic encephalopathy (HE). Despite initial treatment with lactulose and rifaximin, her neurological symptoms worsened, leading to the identification of concurrent acquired hepatocerebral degeneration (AHD). This condition is characterised by cognitive decline, movement disorders and distinctive imaging abnormalities. The discussion highlights the challenges in distinguishing AHD from HE, underscoring the sophisticated diagnostic and management strategies required for such intricate cases in the realm of chronic liver disease.

show abstract

Section: Discussionmentioning

confidence: 99%

Navigating diagnostic challenges: a case of acquired hepatocerebral degeneration presenting as worsening hepatic encephalopathy in chronic liver disease

Raza,

Bzowej,

Rini

et al. 2024

European Journal of Case Reports in Internal Medicine

View full text Add to dashboard Cite

show abstract

“…Whether these changes are mediated through direct conformational changes in the protein structure or indirect mechanisms of ammonia, however, cannot be disentangled with the present findings. Thus, hepatocerebral degeneration ( Victor et al, 1965 ; Yalçın et al, 2016 ) might contribute to the signal reduction in APT w images in more severe HE affecting the cerebellum specifically ( Butterworth, 2007 ). Cerebellar degeneration in HE is characterized by loss of Purkinje cells, and alcoholic abuse is associated with a greater degree of severity of loss of these cells, and a higher HE prevalence ( Kril et al, 1997 ).…”

Section: Discussionmentioning

confidence: 99%

Chemical exchange saturation transfer imaging in hepatic encephalopathy

Zöllner

Butz

Jördens

et al. 2019

NeuroImage: Clinical

View full text Add to dashboard Cite

Hepatic encephalopathy (HE) is a common complication in liver cirrhosis and associated with an invasion of ammonia into the brain through the blood-brain barrier. Resulting higher ammonia concentrations in the brain are suggested to lead to a dose-dependent gradual increase of HE severity and an associated impairment of brain function. Amide proton transfer-weighted (APTw) chemical exchange saturation transfer (CEST) imaging has been found to be sensitive to ammonia concentration. The aim of this work was to study APTw CEST imaging in patients with HE and to investigate the relationship between disease severity, critical flicker frequency (CFF), psychometric test scores, blood ammonia, and APTw signals in different brain regions.Whole-brain APTw CEST images were acquired in 34 participants (14 controls, 20 patients (10 minimal HE, 10 manifest HE)) on a 3 T clinical MRI system accompanied by T1 mapping and structural images. T1 normalized magnetization transfer ratio asymmetry analysis was performed around 3 ppm after B0 and B1 correction to create APTw images. All APTw images were spatially normalized into a cohort space to allow direct comparison. APTw images in 6 brain regions (cerebellum, occipital cortex, putamen, thalamus, caudate, white matter) were tested for group differences as well as the link to CFF, psychometric test scores, and blood ammonia.A decrease in APTw intensities was found in the cerebellum and the occipital cortex of manifest HE patients. In addition, APTw intensities in the cerebellum correlated positively with several psychometric scores, such as the fine motor performance scores MLS1 for hand steadiness / tremor (r = 0.466; p = .044) and WRT2 for motor reaction time (r = 0.523; p = .022). Moreover, a negative correlation between APTw intensities and blood ammonia was found for the cerebellum (r = −0.615; p = .007) and the occipital cortex (r = −0.478; p = .045). An increase of APTw intensities was observed in the putamen of patients with minimal HE and correlated negatively with the CFF (r = −0.423; p = .013).Our findings demonstrate that HE is associated with regional differential alterations in APTw signals. These variations are most likely a consequence of hyperammonemia or hepatocerebral degeneration processes, and develop in parallel with disease severity.

show abstract

“…APT‐weighted imaging is used as a marker for protein content and is altered by conformational changes within intracellular protein compounds . Thus, hepatocerebral degeneration may explain signal reduction in APT‐weighted images with increased concentration of ammonia.…”

Section: Discussionmentioning

confidence: 99%

Ammonia‐weighted imaging by chemical exchange saturation transfer MRI at 3 T

et al. 2018

View full text Add to dashboard Cite

Hepatic encephalopathy (HE) is triggered by liver cirrhosis and is associated with an increased ammonia level within the brain tissue. The goal of this study was to investigate effects of ammonia on in vitro amide proton transfer (APT)-weighted chemical exchange saturation transfer (CEST) imaging in order to develop an ammonia-sensitive brain imaging method. APT-weighted CEST imaging was performed on phantom solutions including pure ammonia, bovine serum albumin (BSA), and tissue homogenate samples doped with various ammonia concentrations. All CEST data were assessed by magnetization transfer ratio asymmetry. In addition, optical methods were used to determine possible structural changes of the proteins in the BSA phantom. In vivo feasibility measurements were acquired in one healthy participant and two patients suffering from HE, a disease associated with increased brain ammonia levels. The CEST effect of pure ammonia showed a base-catalyzed behavior. At pH values greater than 5.6 no CEST effect was observed. The APT-weighted signal was significantly reduced for ammonia concentrations of 5mM or more at fixed pH values within the different protein phantom solutions. The optical methods revealed no protein aggregation or denaturation for ammonia concentrations less than 5mM. The in vivo measurements showed tissue specific and global reduction of the observed CEST signal in patients with HE, possibly linked to pathologically increased ammonia levels. APT-weighted CEST imaging is sensitive to changes in ammonia concentrations. Thus, it seems useful for the investigation of pathologies with altered tissue ammonia concentrations such as HE. However, the underlying mechanism needs to be explored in more detail in future in vitro and in vivo investigations.

show abstract

Acquired hepatocerebral degeneration

Cited by 8 publications

References 9 publications

Navigating diagnostic challenges: a case of acquired hepatocerebral degeneration presenting as worsening hepatic encephalopathy in chronic liver disease

Navigating diagnostic challenges: a case of acquired hepatocerebral degeneration presenting as worsening hepatic encephalopathy in chronic liver disease

Chemical exchange saturation transfer imaging in hepatic encephalopathy

Ammonia‐weighted imaging by chemical exchange saturation transfer MRI at 3 T

Contact Info

Product

Resources

About