Hepatocerebral Degeneration Overview
Learn About Hepatocerebral Degeneration
Hepatocerebral degeneration is a brain disorder that occurs in people with liver damage.
Chronic acquired (Non-Wilsonian) hepatocerebral degeneration; Hepatic encephalopathy; Portosystemic encephalopathy
This condition may occur in any case of acquired liver failure, including severe hepatitis.
Liver damage can lead to the buildup of ammonia and other toxic materials in the body. This happens when the liver doesn't work properly. It does not break down and eliminate these chemicals. The toxic materials can damage brain tissue.
Specific areas of the brain, such as the basal ganglia, are more likely to be injured from liver failure. The basal ganglia help control movement. This condition is the "non-Wilsonian" type. This means that the liver damage is not caused by copper deposits in the liver. This is a key feature of Wilson disease.
Symptoms may include:
- Difficulty walking
- Impaired intellectual function
- Jaundice
- Muscle spasm (myoclonus)
- Rigidity
- Shaking of arms, head (tremor)
- Twitching
- Uncontrolled body movements (chorea)
- Tremor of the hand when the wrist is extended (also called a flapping tremor or asterixis)
- Unsteady walking (ataxia)
Treatment helps reduce the toxic chemicals that build up from liver failure. It may include antibiotics (rifaximin) or a medicine such as lactulose, which lowers the level of ammonia in the blood.
A treatment called branched-chain amino acid therapy may also:
- Improve symptoms
- Reverse brain damage
There is no specific treatment for the neurologic syndrome, because it is caused by irreversible liver damage. A liver transplant may cure the liver disease. However, this operation may not reverse the symptoms of brain damage.
West Virginia University Medical Corporation
Jeffrey Carpenter is a Radiologist and a Neuroradiologist in Martinsburg, West Virginia. Dr. Carpenter and is rated as an Experienced provider by MediFind in the treatment of Hepatocerebral Degeneration. His top areas of expertise are Stroke, Lung Cancer, Brain Aneurysm, Thrombectomy, and Gamma Knife Radiosurgery.
Jason Polchinski is a Neurologist in Martinsburg, West Virginia. Dr. Polchinski and is rated as an Experienced provider by MediFind in the treatment of Hepatocerebral Degeneration. His top areas of expertise are Migraine, Post-Traumatic Epilepsy, Cerebellar Degeneration, and Migraine with Brainstem Aura. Dr. Polchinski is currently accepting new patients.
Winchester Neurological Consultants Full Phreesia
Paul Lyons is a Neurologist and a Sleep Medicine provider in Winchester, Virginia. Dr. Lyons and is rated as an Advanced provider by MediFind in the treatment of Hepatocerebral Degeneration. His top areas of expertise are Seizures, Memory Loss, Stroke, and Generalized Tonic-Clonic Seizure. Dr. Lyons is currently accepting new patients.
This is a long-term (chronic) condition that may lead to irreversible nervous system (neurological) symptoms.
The person may continue to get worse and die without a liver transplant. If a transplant is done early, the neurological syndrome may be reversible.
Complications include:
- Hepatic coma
- Severe brain damage
Contact your health care provider if you have any symptoms of liver disease.
It is not possible to prevent all forms of liver disease. However, alcoholic and viral hepatitis may be prevented.
To reduce your risk of getting alcoholic or viral hepatitis:
- Avoid risky behaviors, such as IV drug use or unprotected sex.
- Don't drink, or drink only in moderation.
Summary: Wilson's disease (WD), also known as Wilson's disease, is a rare autosomal recessive metabolic disorder caused by a mutation of the copper transport ATPase β (ATP7B) gene located on the long arm of chromosome 13 (13q14.3). This leads to accumulation of copper ions in multiple organs such as liver, brain and kidney, resulting in organ involvement. In this study, rAAV8 as the carrier of gene therapy...
Summary: Newborn screening (NBS) is a global initiative of systematic testing at birth to identify babies with pre-defined severe but treatable conditions. With a simple blood test, rare genetic conditions can be easily detected, and the early start of transformative treatment will help avoid severe disabilities and increase the quality of life. Baby Detect Project is an innovative NBS program using a pane...
Published Date: November 02, 2022
Published By: Michael M. Phillips, MD, Emeritus Professor of Medicine, The George Washington University School of Medicine, Washington, DC. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
Garcia-Tsao G. Cirrhosis and its sequelae. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 144.
ul Haq I, Liebenow B, Okun MS. Clinical overview of movement disorders. In: Winn HR, ed. Youmans and Winn Neurological Surgery. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 105.