Wernicke encephalopathy

The Wernicke encephalopathy is a systemic degenerative brain disease based on a vitamin B1 deficiency. Alcoholics, patients with eating disorders or people with chronic intestinal disorders are particularly affected by the disease. The treatment is anchored in a substitution of the missing thiamine.

What is Wernicke encephalopathy?

Encephalopathies are disorders that affect the brain as a whole. For example, they can be caused by degenerative diseases. Whole brain damage can, however, also be systemic and thus be related to cardiopulmonary, renal, hepatic or endocrine diseases that have metabolic consequences for the brain.

The brain depends on various substances. These substances include vitamin B1, also known as thiamine. A particularly large amount of thiamine is required in certain regions of the brain, as it maintains the activity of the ion channels in axons. In addition to the causes mentioned, encephalopathy can also be caused by a lack of thiamine.

Encephalopathy caused in this way is referred to as degenerative Wernicke encephalopathy or that Wernicke-Korsakoff Syndromethat usually affects adults. The first description of the disease goes back to C. Wernicke, who first described the disease in three alcoholics in the 19th century.

causes

The primary cause of Wernicke encephalopathy is hypovitaminosis. This vitamin deficiency can be due, for example, to chronic alcohol abuse. Other common associations are eating disorders, bariatric surgery, malnutrition, chronic intestinal diseases with diarrhea and vomiting, or chemotherapy.

With hypovitaminosis, there is a vitamin B1 deficiency, which is also known as thiamine deficiency. Thiamine is essential as a cofactor for the intermediate metabolism, for example for processes like ketoglutarate dehydrogenase, transketolase or pyruvate dehydrogenase. The intermediate metabolism is severely impaired by a vitamin B1 deficiency. The energy metabolism is damaged and cells perish.

Because of the cell death, Wernicke encephalopathy is to be understood as a neurodegenerative disease and primarily affects areas of the brain with a high thiamine requirement. There is a particularly high demand for Corpora mamillaria in front of, but also the area around the third cerebral ventricle, the nuclei of the thalamus, which Corpora geniculata or the aqueduct area may be affected.

Symptoms, ailments & signs

In the acute course of Wernicke encephalopathy, a reddish-brown discoloration can be seen macroscopically in the thiamine-requiring brain regions. Multiple petechial hemorrhages can be seen. With a chronic course, atrophy of the corpora mamillaria sets in. The histological picture is characterized by ganglion cell losses.

Under the microscope, spongy nerve loosening can occur, which is characterized by glial and vascular proliferation with siderophages. From a clinical point of view, a classic triad consists of impaired consciousness or disorientation, gait ataxia and disorders of the eye muscles. Usually there is an organic brain psychosyndrome, which is characterized by cognitive disorders. Intellectual degradation with memory loss can also be a characteristic symptom.

In addition to the ocular muscle paresis, there may also be nystagmus of the eyes. Additional symptoms can be polyneuropathies, reflex disorders and dysdiadochokinesis. Dysphagia, dysarthria or vegetative disorders such as hypotension, hypothermia or hyperhidrosis are just as common. Which individual disorders are present depends on the individual case and the affected brain regions. Also, the cause in question can vary the clinical picture to a greater or lesser extent in individual cases.

Diagnosis & course of disease

The suspected diagnosis of Wernicke syndrome is made by the doctor with the anamnesis and the first impression of the patient's clinical picture. In the anamnesis, previously diagnosed intestinal diseases, eating disorders or alcohol problems can be an important clue. To establish a connection with vitamin deficiency, the vitamin B1 level in the blood is detected.

The plasma level can produce false negative values. Therefore, a more sensitive [[[whole blood test]] is becoming the diagnostic of choice. An imaging method is usually used to determine the localization of brain lesions. Both CT and MRI are suitable methods. The course of the disease depends on the primary cause of the vitamin deficiency.

A chronic bowel disease, for example, usually has a more unfavorable course than a current acute bowel disease with only temporary diarrhea and vomiting. In the case of Wernicke encephalopathy following alcohol abuse or eating disorders, the course depends entirely on the patient's cooperation. If left untreated, the disease can be fatal.

Complications

Wernicke encephalopathy has a very negative effect on the patient's consciousness and can thus lead to various serious complaints. As a rule, those affected suffer from impaired consciousness and can no longer cope with everyday life on their own. Concentration and coordination are disturbed, so that patients are usually always dependent on the help of other people.

This also prevents the ingestion of food and liquids. Often there is memory loss and various cognitive disorders that have a negative impact on the patient's quality of life. Most sufferers also lose consciousness of falling into a coma.

The severity of the symptoms of Wernicke encephalopathy depends heavily on their exact cause, so that no general prediction can be made here. In the treatment, however, the treatment of the underlying disease is always necessary first. The symptoms of the disease can be limited in some cases.

However, a completely positive course of the disease is not achieved. The patient's relatives are often affected by psychological complaints and therefore also need psychological treatment.

When should you go to the doctor?

Changes and abnormalities in brain activity should always be examined and clarified by a doctor. If eating disorders, diarrhea or vomiting occur, health check-ups are advisable. Deficiency symptoms, changes in mental or physical performance and disorientation must be examined as quickly as possible. In the event of a disturbance of consciousness or loss of consciousness, an ambulance service must be alerted.

There is an acute health-threatening situation in which intensive medical care is necessary. A doctor is required in the event of excessive alcohol consumption, reduced physical strength or persistent or increasing feeling of illness. If the person concerned refuses to eat or if he is unable to stop consuming alcohol on his own responsibility, he must seek the help of a doctor as soon as possible.

If there are speech disorders, states of confusion or involuntary twitching of the muscles, the person concerned needs medical support. Since the Wernicke encephalopathy is fatal if left untreated, a doctor should be consulted as soon as the first irregularities appear. If the person concerned can no longer perceive the obligations of everyday life, if behavioral problems and changes in personality appear, this is extremely worrying. A doctor is needed to clarify the cause.

Therapy & Treatment

Wernicke encephalopathy is treated with a different focus depending on the primary cause. In the case of alcohol abuse as the primary cause, for example, absolute abstinence from alcohol is the therapy recommendation of choice. The doctor must make the patient clearly aware of the context of his illness and usually works with a psychotherapist. Closed or open alcohol therapy is often the only way to achieve lasting improvement.

Closed or open therapies are also the treatment option for eating disorders. In order to alleviate Wernicke encephalopathy as a symptom of eating disorders or alcohol addiction in an acute phase, high doses of thiamine are administered parenterally. The absorption of oral thiamine is variable and difficult to control. Therefore, intravenous administration is the more sensible treatment method in emergency situations. Usually about 200 milligrams of thiamine is administered over two days.

A dose of 500 milligrams three times a day over two days is also possible. After completing these measures, long-term oral administration over a certain period is recommended. In addition, on the part of drug therapy, the administration of magnesium is often added. Chronic intestinal patients usually require a life-long substitution of thiamine.

For patients with malnutrition symptoms, an informative nutrition plan is ideally created. Nutrition plans can be useful in connection with the treatment of Wernicke encephalopathies.

prevention

Wernicke encephalopathy can be prevented to a certain extent through a balanced diet and responsible use of alcohol. As a secondary disease of various intestinal diseases, these measures cannot prevent the disease in every case.

Aftercare

Follow-up treatment for Wernicke encephalopathy depends on the underlying disease. Since there is a deficiency in vitamin B-1 in all cases of Wernicke encephalopathy, a renewed vitamin B-1 deficiency must be avoided. For this purpose, vitamin B-1 should be taken as a precautionary measure in moderate doses as a dietary supplement in addition to daily food intake.

In addition, the vitamin B-1 level in the blood must be checked regularly in order to detect a renewed deficiency at an early stage. If a chronic inflammatory bowel disease (Crohn's disease) has led to the development of the vitamin B-1 deficiency, this must be treated permanently and causally. If alcohol is responsible for the development of the vitamin B-1 deficiency and the subsequent Wernicke encephalopathy, alcohol withdrawal must take place.

An inpatient stay in a withdrawal clinic may be necessary for this. If chemotherapy has led to the development of the disease, the vitamin B-1 intake must be increased. Since food supplements are often not sufficient for chemotherapy, this can also be done intravenously via infusions.

If the cause of the underlying vitamin B-1 deficiency cannot be clearly identified, various gastroenterological (colonoscopy) and endocrinological examinations (hormone tests) must be carried out in order to determine and treat them. In addition, regular neurological examinations of motor skills, sensory functions, reflexes and coordination as well as brain imaging (MRT, CT) should be carried out in order to identify and treat long-term damage to nerves and brain at an early stage.

You can do that yourself

The possibilities for self-help are very limited in the event of an outbreak of Wernicke encephalopathy. There is a previous illness that needs to be treated and treated. Mostly it is an addiction disease or a health disorder with a chronic course. The person affected should clarify and remedy the causes of the underlying disease as best possible. This is only possible with difficulty, since he is usually already in an advanced stage of the disease at hand.

Nevertheless, the consumption of harmful substances should be minimized under all circumstances. The consumption of alcohol is to be completely avoided if possible. Addicts rarely manage this step on their own. It is therefore advisable to work with a doctor and a psychotherapist.

The motivation to change is the basic requirement so that an improvement in the overall situation can be seen. Strengthening general wellbeing and setting life goals that are to be achieved are just as important. This procedure supports changes in behavior and contributes significantly to alleviating existing complaints.

Targeted training and exercise units help improve concentration. These can be implemented independently at any time and help with memory activity. In addition, a healthy and balanced diet is important to support the functioning of the organism.