الفهرس 
ANATOMY & PHYSIOLOGY OF BASAL GANGLIA AND LIVEROnly 14 pages are availabe for public view
 |  | from | 139 |  |  |
| | | from | 139 | | |
Abstract
The liver is the largest internal organ of the body. The liver performs many functions. These essential functions are impaired when a liver develops cirrhosis. Estimated cirrhosis mortality is between 34 - 66%, largely dependent on the cause of the cirrhosis; alcoholic cirrhosis has a worse prognosis than primary biliary cirrhosis and cirrhosis due to viral hepatitis.
Liver cirrhosis increases resistance to blood flow and higher pressure in the portal venous system, resulting in portal hypertension. Portal hypertension leads usually to variceal formation and emergence of shunts between portal and systemic circulation, which are accused to be the main reason for extrapyramidal symptoms.
Hepatic encephalopathy is a syndrome of neurological and psychiatric disturbances considered as a hallmark of acute hepatic failure. Hepatic encephalopathy in cirrhotic patients progresses from sleep disturbances and slight attention deficits to disorientation, disturbed consciousness and perhaps coma over time periods ranging from several days up to months. Patients with acute liver failure may present with altered behavior even before the first clinical signs of altered liver function are present.
A minority of patients with chronic liver disease develop acquired form of hepatocerebral degeneration. This is attributed to prolonged exposure to metabolic toxins that cause encephalopathy, especially manganese. Dementia, dysarthria, cerebellar dysfunction, movement disorders, rigidity and myelopathy have all been reported. Hepatocerebral degeneration includes acquired and familial causes; both can have similar clinical presentations. In the inherited form there is a metabolic dysfunction which leads to copper deposition in neurons. Consequently, the disease causes Parkinsonism, dystonia, and abnormal movements that include athetosis and chorea. In the acquired form, akinetic-rigid symptoms were prevalent and are caused mainly by manganese deposition in neurons. Recent neuroradiological imaging studies mainly brain MRI; have shown hyperintense signals in various brain regions on T1 weighted images.
The current study aimed to determine the prevalence of extrapyramidal signs in chronic liver disease patients. As to our knowledge, there is no available data about the prevalence of extrapyramidal signs in chronic liver disease patients in Egypt.
All patients admitted in El-Demerdash Hospital, Department of Tropical Medicine were asked to participate in the study. Five months were included in this study; starting from August 2014 – November 2014. 53 patients were excluded. The total number of patients included all over the five months was 118 patients. The age of the patients ranged from 20 to 76 years, with mean age of 53.29 years old.
Data related to the diagnosis of chronic liver disease and its complications were collected. That included laboratory investigations, imaging studies and screening neurological examination and assessment that was done by the main researcher and audited by a senior staff member, Dr. Ahmed Gaber, Professor of Neurology, Ain Shams University, to assess the patients for extrapyramidal signs.These signs were quantified by Fahn-Marsden (BFM) Scale & Unified Parkinson Disease Rating Scale (UPDRS).
The main findings of the current study were that 70 patients (59.3 %) of the studied sample had parkinsonian features at the time of assessment, most commonly found in those patients was body bradykinesia, found among 56 patients (80%). Of those 70 patients, 51 patients (72.8%) showed axial distribution of features. Only two patients (1.7%) among the studied sample showed focal leg dystonia at the time of assessment.
The majority of the patients studied had dilated portal veins (52.5%). A significant relation was found between portal vein diameter and presence of parkinsonian features among the studied sample.
Another finding in the current study was a weak positive correlation was found between age and the total score of UPDRS. Moreover, a weak positive correlation was found between INR and total bilirubin with the total score of UPDRS. Another moderate positive correlation was found between the number of hepatic encephalopathies and the total score of UPDRS, whereas a weak negative correlation was found between serum albumin with the total score.
Interestingly, a strong positive correlation was found between serum potassium and the total score of UPDRS in the current study.
To our knowledge, the present study is the first to describe the relation between HCV status and dilated portal vein on one hand and the axial distribution of parkinsonian features on the other hand.Moreover, a positive correlation was found between age of onset and number of hepatic encephalopathies with the axial sub-score of UPDRS among the studied sample. On the contrary to the above findings related to the total score, there was no significant correlation between the axial sub-score and other variables including total bilirubin, INR and serum albumin.
Finally, an interesting finding of the current study was that patients who had parkinsonian features showed a significant relation between positive HCV status in particular and axial distribution of their features, which is hard to explain and may indicate that the effect of etiology is more complex and not straight forward. This may need further in-depth studies.