A Rare Neurological Complication in Advanced Liver Disease: Acquired Hepatocerebral Degeneration

2021 ◽  
Author(s):  
Minnie Au ◽  
Claire Smart ◽  
Mohamed Asif Chinnaratha
Keyword(s):  
Liver Disease ◽  
Neurological Complication ◽  
Advanced Liver Disease ◽  
Acquired Hepatocerebral Degeneration ◽  
Hepatocerebral Degeneration

Acquired hepatocerebral degeneration (AHD): a peculiar neurological impairment in advanced chronic liver disease

2017 ◽  
Vol 33 (1) ◽  
pp. 347-352 ◽  
Author(s):  
A. Pigoni ◽  
F. Iuculano ◽  
C. Saetti ◽  
L. Airaghi ◽  
L. Burdick ◽  
...  
Keyword(s):  
Liver Disease ◽  
Chronic Liver Disease ◽  
Neurological Impairment ◽  
Chronic Liver ◽  
Acquired Hepatocerebral Degeneration ◽  
Hepatocerebral Degeneration

scholarly journals Acquired Hepato Cerebral Degeneration – An Atypical Finding in MRI of Brain mimicking Bilateral Ischaemic Stroke

2016 ◽  
Vol 4 (1) ◽  
pp. 54-57
Author(s):  
Abdul Kader Shaikh ◽  
Mahjabin Islam ◽  
Ahijit Chowdhury ◽  
Ashraf Uddin Ahmed ◽  
Utpal Dasgupta
Keyword(s):  
Ischemic Stroke ◽  
Case Report ◽  
Liver Disease ◽  
Basal Ganglia ◽  
Liver Failure ◽  
Common Condition ◽  
Acquired Hepatocerebral Degeneration ◽  
Abnormal Movements ◽  
Hepatocerebral Degeneration ◽  
Cerebral Degeneration

Acquired Hepatocerebral Degeneration (AHD) produces a panel of neurological symptoms occurring in patients with chronic liver disease. These symptoms may mimic different neurological abnormalities e.g. abnormal movements, rigidity, parkinsonism, ataxia, neuropsychiatric and cognitive manifestations.[1] Acquired Hepatocerebral Degeneration is usually encountered in patients with CLD due to accumulation of manganese & other metabolic toxins in basal ganglia, following repeated episodes of liver failure[2] .The aim of this case report is to highlight the MRI finding of Acute Hepatocerebral Degeneration which often can be underappreciated owing to the possibility of diagnosing a relatively common condition that is Bilateral Ischemic stroke.[19].Bangladesh Crit Care J March 2016; 4 (1): 54-57

Disorders of Manganese Metabolism

2016 ◽  
Author(s):  
Karin Tuschl ◽  
Peter T. Clayton ◽  
Philippa B. Mills
Keyword(s):  
Liver Disease ◽  
Childhood Onset ◽  
Acquired Hepatocerebral Degeneration ◽  
Liver Disease Progression ◽  
Lower Blood ◽  
Hepatocerebral Degeneration ◽  
A Cell ◽  
End Stage ◽  
Blood Manganese

Manganese is an essential trace metal for numerous metalloenzymes. Manganese homeostasis requires tight regulation in vivo and disruption of this balance can lead to manganese overload and subsequent accumulation of manganese in brain, liver, and blood. Mutations in SLC30A10, a cell surface-localized manganese efflux transporter, cause an autosomal recessive hypermanganesemia syndrome with two distinct phenotypes: childhood onset dystonia and adult onset Parkinsonism, associated with chronic liver disease, polycythemia and features of iron depletion. MRI brain appearances are characteristic of Mn deposition with hyperintense basal ganglia on T1-weighted images. Chelation therapy with disodium calcium edetate and iron supplementation effectively lower blood manganese levels, halt liver disease progression and improve neurological symptoms.The inherited form of hypermanganesemia can be distinguished from acquired causes of manganese overload including environmental overexposure and acquired hepatocerebral degeneration in cases of end stage liver disease.

scholarly journals Acquired hepatocerebral degeneration and hepatic encephalopathy: correlations and variety of clinical presentations in overt and subclinical liver disease

2011 ◽  
Vol 69 (3) ◽  
pp. 496-501 ◽  
Author(s):  
Fernando G. Romeiro ◽  
Madileine F. Américo ◽  
Fábio S. Yamashiro ◽  
Carlos A. Caramori ◽  
Arthur O. Schelp ◽  
...  
Keyword(s):  
Liver Disease ◽  
Hepatic Encephalopathy ◽  
Hepatolenticular Degeneration ◽  
Acquired Hepatocerebral Degeneration ◽  
Flow Interruption ◽  
Hepatic Diseases ◽  
Portosystemic Shunts ◽  
Clinical Presentations ◽  
Hepatocerebral Degeneration ◽  
Similar Motor

Acquired hepatocerebral degeneration (AHD) and hepatolenticular degeneration can have similar clinical presentations, but when a chronic liver disease and atypical motor findings coexist, the distinction between AHD and hepatic encephalopathy (HE) can be even more complicated. We describe three cases of AHD (two having HE) with different neuroimaging findings, distinct hepatic diseases and similar motor presentations, all presenting chronic arterial hypertension and weight loss before the disease manifestations. The diagnosis and physiopathology are commented upon and compared with previous reports. In conclusion, there are many correlations among HE, hepatolenticular degeneration and AHD, but the overlapping of AHD and HE could be more common depending on the clinical knowledge and diagnostic criteria adopted for each condition. Since AHD is not considered a priority that affects the liver transplant list, the prognosis in AHD patients remains poor, and flow interruption in portosystemic shunts must always be taken into account.

Acquired hepatocerebral degeneration without overt liver disease

Neurology ◽  
2004 ◽  
Vol 63 (10) ◽  
pp. 1981-1982 ◽  
Author(s):  
M. A.C. Saporta ◽  
C. Andre ◽  
P. R.V. Bahia ◽  
L. Chimelli ◽  
P. H.N. Pais ◽  
...  
Keyword(s):  
Liver Disease ◽  
Acquired Hepatocerebral Degeneration ◽  
Hepatocerebral Degeneration

Implications of Manganese in Chronic acquired hepatocerebral degeneration

2018 ◽  
Vol 17 (5) ◽  
pp. 0-10
Author(s):  
Neil Rajoriya ◽  
Mayur Brahmania ◽  
Jordan J Feld
Keyword(s):  
Liver Cirrhosis ◽  
Liver Disease ◽  
Neurological Diseases ◽  
Rare Condition ◽  
Severe Form ◽  
Neurological Manifestations ◽  
Acquired Hepatocerebral Degeneration ◽  
Hepatocerebral Degeneration ◽  
Neurological Presentation ◽  
The Brain

Neurological symptoms can be one of the over-riding symptoms in patients with liver cirrhosis. Patients can present with subtle changes in mood or neurological function due to hepatic encephalopathy (HE), to more severe presentations including stupor and coma. While HE, in its severe form, can be clinically easy to diagnose, more subtle forms may be more difficult to recognize. Other neurological diseases may indeed be overlooked in the context of cirrhosis or confuse the physician regarding the diagnosis. Chronic acquired hepatocerebral degeneration (CAHD) is an uncommon problem occurring in patients with cirrhosis characterised by a Parkinsonian-like neurological presentation with damage to the brain secondary to manganese (Mn) deposition. Here we describe a case of a patient with a neurological presentation of liver disease with a review of the current CAHD literature. In conclusion, CAHD is a rare condition occurring in liver cirrhosis that should always be considered in patients with neurological manifestations of chronic liver disease.

scholarly journals Acquired hepatocerebral degeneration: a case report

2012 ◽  
Vol 6 (1) ◽  
pp. 59-63 ◽  
Author(s):  
Clarice Listik ◽  
Gislaine Cristina Lopes Machado-Porto ◽  
Maira Okada de Oliveira ◽  
Fábio Henrique de Gobbi Porto
Keyword(s):  
Case Report ◽  
Liver Disease ◽  
Basal Ganglia ◽  
Cognitive Decline ◽  
Chronic Liver Disease ◽  
Movement Disorders ◽  
Cognitive Symptoms ◽  
Acquired Hepatocerebral Degeneration ◽  
Portosystemic Shunting ◽  
Hepatocerebral Degeneration

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.

Serum CYFRA 21 – 1 levels are elevated in advanced liver disease and associate with poor survival

2019 ◽  
Author(s):  
K Hamesch ◽  
N Guldiken ◽  
M Aly ◽  
N Hueser ◽  
D Hartmann ◽  
...  
Keyword(s):  
Liver Disease ◽  
Poor Survival ◽  
Advanced Liver Disease
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