OVERVIEW
Acquired hepatocerebral degeneration (AHCD) is a clinicopathologic syndrome of irreversible neurodegeneration caused by chronic liver disease that triggers brain dysfunction. It occurs in a wide range of chronic liver diseases, among which it is more common in alcoholic cirrhosis, subacute and chronic hepatitis, and after portal vein shunt surgery. Pathologic changes in this disease are mainly Alzheimer type II astrocyte proliferation and diethylstilbestrol deficiency.
Etiology
1. The etiology and pathogenesis of this disease are not fully understood, and studies have suggested that it is related to spontaneous porto-caval shunt originating from the basis of liver disease or hepatic porto-caval shunt surgery.
2. In addition, the clinical manifestations of acquired hepatic encephalopathy are similar to those of manganese poisoning in the early stage of neurological damage, and the concentration of manganese in serum, cerebrospinal fluid and brain tissue of patients with cirrhosis is obviously increased, and the content of manganese can be up to 3-9 times of the normal value in the case of hepatic failure; the magnetic resonance imaging of brain in the patients with acquired hepatic encephalopathy showed Tl high-density shadow, which is in line with the imaging manifestations of occupational manganese intoxication, suggesting that manganese may play an important role in the occurrence of acquired hepatic encephalopathy. This suggests that manganese may play an important role in the development of acquired hepatic encephalopathy.
Symptoms
Clinical manifestations are cognitive and behavioral changes, including marked slowness of thought, inattention, and emotional apathy. Motor abnormalities may be present, including dystonia, chorea, parkinsonism, ataxia, and stereotyped speech, which behave similarly to delayed-onset movement disorders.
Examination
1. Laboratory examination
Serum, cerebrospinal fluid and brain tissue manganese concentration is obviously elevated in patients with cirrhosis, and the manganese content in liver failure can be up to 3-9 times of the normal value. Patients with chronic liver disease often have increased blood ammonia.
2.Imaging examination
Magnetic resonance imaging is the best, and the typical manifestation is bilateral basal ganglia T1W high signal.
3. Histopathologic examination
The main pathological changes are Alzheimer type II astrocyte proliferation and diethylstilbestrol deficiency. Laminar necrosis or pseudolaminar necrosis of the gray-white matter junction, striatum, and cerebellar white matter with tiny cystic cavities can be seen.
Diagnosis.
The patient had a history of liver disease, and the diagnosis was clear based on the clinical presentation and the results of related tests such as magnetic resonance imaging of the brain.
Differential diagnosis
This disease should be differentiated from hepatic encephalopathy, alcoholic cerebral degeneration and idiopathic Parkinson’s disease.
1. Hepatic encephalopathy
Unlike hepatic encephalopathy, the motor abnormalities in acquired hepatic encephalopathy do not improve with recovery of consciousness and are not sensitive to blood ammonia-lowering therapy.
2. Idiopathic Parkinson’s disease
Acquired hepatic encephalopathy-associated Parkinson’s disease and idiopathic Parkinson’s disease can both manifest as motor tremor, rigidity, flaccidity, postural instability and trailing gait, but the former manifests bilateral symmetrical dyskinesia in the early stage of the disease, which progresses rapidly, and most of the patients are unresponsive to dopamine therapy.
3. Alcoholic brain degeneration
Acquired hepatic encephalopathy and alcoholic encephalopathy can both manifest ataxia. Acquired hepatic encephalopathy is mainly characterized by motor disorders of the limbs, and the motor disorders appear only after a long latency period after abstinence from alcohol, and the brain magnetic resonance imaging shows T1W high signal, and the above features can be distinguished from alcoholic encephalopathy.
Treatment
Primary liver disease should be treated early. Once clinical symptoms appear, the following measures can be taken.
1. Limit manganese intake
Manganese-rich foods such as tea, fruits, cereals, nuts and beans should be prohibited.
2. Manganese repellent treatment
Oral treatment of tretinoin, which can be chelated with manganese ions to facilitate their excretion from the body, is effective for some patients with acquired hepatic encephalopathy-associated Parkinson’s Disease.
3. Tetrabenazine
A presynaptic dopamine depleting drug, used in the United States for the treatment of acquired hepatic encephalopathy-associated chorea and orofacial dyskinesia, with recent efficacy in some patients.
4. Liver transplantation
Liver transplantation early in the course of the disease may relieve symptoms, but is ineffective in long-term severe neurologic damage.
5. Other
Oral lactulose and antibiotics, short-term restriction of protein intake.
Prognosis
The neurodegeneration in acquired hepatic encephalopathy is irreversible and the motor abnormalities do not improve with recovery of consciousness.