Encephalopathy: Causes, Symptoms & Treatment

Encephalopathy characterizes pathological conditions of the brain caused by various causes. The symptomatology of brain dysfunction is independent of the underlying diseases. At least initially, no structural changes occur in the brain, so once the causes of the neurologic deficits are corrected, the symptoms can often resolve.

What is encephalopathy?

Encephalopathy is a collective term for pathological processes in the brain that are not due to brain-organic structural changes. This means that external influences such as poisoning, viruses, prions, or hypertension initially disrupt certain functional processes in the brain. However, it is difficult to clearly distinguish encephalopathies from other diseases of the brain. For example, according to this definition, inflammatory processes in the brain do not belong to encephalopathies. Furthermore, in an encephalopathy not only parts of the brain, but the entire brain, are subject to functional disorders. The cause of the occurring symptoms in this disease complex are dysfunctions in the interaction of nerve and glial cells. Internal changes in the organism cause disturbances of the cerebral balance, as a result of which the functions of neurotransmitters and membranes are impaired. Generally, the processes are reversible after the underlying processes have ceased. However, long-term damage may result from inflammatory processes that develop as a consequence of the dysfunctions.

Causes

Causes of encephalopathies may include elevated concentrations of potentially toxic substances from metabolic processes or poisoning, electrolyte disturbances, pathogens, or circulatory disturbances. This is true, among other things, when toxic substances accumulate in the blood as a result of certain organ damage and can no longer be broken down. A well-known example is hepatic encephalopathy. In hepatic encephalopathy, the liver can no longer perform its detoxification function. Thus, in cirrhosis of the liver, the concentration of ammonia in the blood increases because the ammonia derived from protein breakdown can no longer be adequately converted into urea. In the brain, ammonia alters the concentration of certain messenger substances, so that communication between the various nerve and glial cells is disturbed. The cause is a swelling of the astrocytes due to the influence of ammonia. A brain edema develops, which impairs neurotransmitter function. Encephalopathies resulting from toxic influence also include uremic encephalopathy, bilirubin encephalopathy, and dialysis encephalopathy. In uremic encephalopathy, kidney failure is the underlying cause. The kidneys are no longer able to remove urinary substances such as uric acid or creatinine from the blood. These substances disrupt the function of nerve cells in the brain. Bilirubin encephalopathy, in turn, is caused by an increased concentration of unconjugated bilirubin in the blood. This disease mainly affects newborns with severe neonatal jaundice. Dialysis encephalopathy is thought to be primarily due to aluminum intoxication from the use of dialysis fluids containing aluminum. Other forms of encephalopathy include hypertensive encephalopathy, bovine spongiform encephalopathy (BSE, Creutzfeldt-Jakob disease), HIV encephalopathy, Wernicke’s encephalopathy, Hashimoto’s encephalopathy, MELAS syndrome, and Binswanger’s disease. Hypertensive encephalopathy is caused by sudden elevation of arterial blood pressure. Creutzfeldt-Jakob disease is thought to be caused by so-called prions, which were first detected in the bovine brain. Wernicke’s encephalopathy is caused by hypovitaminosis with vitamin B1, which is caused by malnutrition or excessive alcohol consumption. Hashimoto’s encephalopathy is caused by autoimmune processes directed against the brain. MELAS syndrome is a mitochondrial disorder. Binswanger’s disease, in turn, is an encephalopathy caused by arteriosclerosis.

Symptoms, complaints, and signs

Encephalopathies are characterized by rapid behavioral changes. Cognitive and motor slowing occur. Furthermore, disturbances of consciousness from mild drowsiness to coma occur.In addition, there are drive, orientation, attention and memory disorders. Sometimes the patient also suffers from hallucinations and delusions. The overall picture also includes such symptoms as tremor, paralysis, speech disorders, visual disturbances or even epileptic seizures. Vegetative symptoms such as cardiac arrhythmias, breathing difficulties or temperature regulation disorders as well as blood pressure changes may also occur. Not all symptoms have to appear. In many cases, combinations of individual symptoms are observed.

Diagnosis

Based on the symptoms, the cause of the present encephalopathy cannot yet be determined. To do so, it is first important to take a comprehensive history of the patient’s medical history. Of course, other symptoms must also be considered to diagnose the underlying condition. Laboratory tests can determine possible toxins or pathogens. Imaging studies do not yet identify brain organic changes in encephalopathies. Differential diagnosis must be used to delineate other diseases of the central nervous system, such as strokes, trauma, infections, or epilepsies.

Complications

Encephalopathy can have a wide variety of causes, so different complications are possible. On the one hand, the disease of the brain results in a wide variety of paralysis symptoms, as well as convulsions or sensory disturbances. On the other hand, encephalopathy can be caused by an increased concentration of ammonia as is the case, for example, with a failure of the kidney (renal insufficiency). This can end in a life-threatening coma. In addition, renal insufficiency leads to reduced excretion of potassium (hyperkalemia), which promotes the development of cardiac arrhythmias. Fewer acids are also excreted, which also leads to an increase in the concentration of potassium in the blood. Kidney failure also leads to the development of painful edema, especially in the leg area. Cirrhosis of the liver, as occurs with increased alcohol consumption, also leads to the development of encephalopathy. Due to this, less proteins are produced for the body, it is increased the probability of the development of edema and ascites. In addition, disorders in blood coagulation are also conceivable. Likewise, blood flowing through the liver is also diverted and directed to the spleen, which consequently enlarges. In addition, there is also the development of hemorrhoids and varicose veins in the area of the stomach and esophagus, which in the worst case can burst and lead to internal bleeding.

When should you go to the doctor?

A visit to the doctor is necessary as soon as the affected person behaves conspicuously. In addition to unusual changes in behavior, disturbances in consciousness are considered of particular concern. If there is a feeling of lightheadedness, a decrease in general performance or general weakness, consultation with a physician is recommended. If dysfunction of individual systems occurs, a physician is needed to determine the cause and initiate measures of relief. If there are signs of paralysis, numbness of the skin or sensory disturbances, a doctor should be consulted. Decreases in vision, hearing or speech are considered unusual and should be evaluated by a physician as soon as possible. Disturbances of the heart rhythm, palpitations, high blood pressure or a general feeling of malaise must be medically examined and treated. In case of breathing difficulties or interruptions of breathing, a doctor is needed. A life-threatening condition is imminent and should be examined and clarified in time. If orientation disorders, attention deficits or memory problems set in, a doctor must be consulted. If hallucinations or delusions occur, a physician is also needed. Changes in personality, emotional abnormalities or mood swings should be presented to a doctor. A doctor’s visit is also needed if there are epileptic seizures or a general formation of convulsions in the body. Sensations of pain, a diffuse feeling of illness, or unusual listlessness should be discussed with a physician.

Treatment and therapy

Treatment of encephalopathy depends on the underlying cause. In hepatic encephalopathy, therapy of the liver disease is the primary treatment. To alleviate the symptoms of encephalopathy, ammonia concentrations must be decreased.This can be achieved by, among other things, balancing the metabolism, accelerating the urea cycle by administration of ornithine aspartate, by administration of the laxative lactulose, and by administration of antibiotics to reduce ammonia-producing bacteria. Dialysis is indicated in cases of renal failure. If hypovitaminosis with vitamin B1 is present, thiamine (vitamin B1) must be administered in high doses. Furthermore, absolute abstinence from alcohol is necessary. In blood pressure-related encephalopathies, normalization of blood pressure is the primary goal.

Outlook and prognosis

The prognosis of encephalopathy depends on the underlying cause, the progress of the disease, and the patient’s general health. In severe cases, organ failure results in death. Hepatic encephalopathy is reversible with early treatment and good therapy. Symptoms are treated individually until relief begins. Clinically manifest hepatic encephalopathy is likely to have an episodic or chronic course. Each new episode results in a worsening of overall health. In a chronic course, there is continuous deterioration. In both courses of the disease, an increased risk of mortality is to be expected. There is also a risk of coma. If the patient awakens from this coma, severe health impairments are to be expected. A complete recovery is not to be expected. If the patient has Wernicke’s encephalopathy, the progress of the disease is also decisive for the prognosis. With immediate treatment, significant relief of existing symptoms can be achieved. Within a few weeks, there is an improvement in speech or motor disturbances. In about 40% of cases, permanent impairments remain. These have a significant impact on the quality of life. ¾ of all patients suffer psychological sequelae. Patients are often dependent on lifelong support or care.

Prevention

The risk of encephalopathy can generally be significantly reduced by a healthy lifestyle that includes a balanced diet, plenty of exercise, and avoidance of excessive alcohol consumption. Many underlying diseases can be prevented in this way.

Follow-up

In most cases, very few, if any, direct measures and options for aftercare are available to those affected by encephalopathy. In this regard, the disease must primarily be detected very early and subsequently treated to prevent further discomfort or complications in the life of the affected person. An early diagnosis with subsequent treatment always has a positive effect on the further course of the disease and can prevent a further worsening of the symptoms. As a rule, self-healing cannot occur. In most cases, patients with encephalopathy are dependent on taking medication. Antibiotics are prescribed in particular. These should always be taken exactly as prescribed by the doctor in order to alleviate the symptoms. Alcohol should be avoided in order not to reduce the effect of the antibiotics. Furthermore, a healthy lifestyle with a healthy diet always has a positive effect on the further course of encephalopathy. Patients should regularly check their blood pressure and, if necessary, lower it to normal. Whether encephalopathy reduces life expectancy cannot be predicted in general.

Here’s what you can do yourself

Encephalopathy is a collective term for abnormal conditions of the brain that are triggered by a variety of causes. Whether and what a patient can do himself to improve his condition depends on the underlying disease to which the encephalopathy is attributable. Brain disorders, for example, can be triggered by high blood pressure. In this case, the patient can take a number of self-help measures. In addition to regular monitoring of blood pressure, a change in lifestyle and consumption habits is usually unavoidable. Excess weight is a central risk factor, which is why sufferers with too high a body mass index (BMI) must first lose weight permanently.Permanent weight reduction usually requires a change in eating habits, which those affected usually cannot manage without outside support. Patients should therefore consult not only a doctor but also a nutritionist and, if they lack motivation, join a self-help group. Patients can also do much to improve brain disorders caused by hypovitaminosis, for example a deficiency of thiamine (vitamin B1). For example, through a healthy diet and, if still necessary, the use of dietary supplements. Insofar as the vitamin deficiency is caused by abuse of alcohol or other drugs, the patient should begin a rehab course along with accompanying therapy.