Wernicke encephalopathy is a systemic degenerative brain disease based on vitamin B1 deficiency. The disease particularly often affects alcoholics, patients with eating disorders, or those with chronic bowel disease. Treatment anchors in a substitution of the missing thiamine.
What is Wernicke’s encephalopathy?
Encephalopathies are damage that affects the brain as a whole. They can be caused by degenerative diseases, for example. However, whole-brain damage can also be systemic and thus related to cardiopulmonary, renal, hepatic, or endocrine diseases that have metabolic consequences for the brain. The brain is dependent on various substances. Among these substances is vitamin B1, also known as thiamine. Certain brain regions require particularly high levels of thiamine because it maintains ion channel activity in axons. An encephalopathy can therefore be caused by a deficiency of thiamine in addition to the aforementioned causes. An encephalopathy caused in this way is referred to as a degenerative Wernicke encephalopathy or Wernicke-Korsakow syndrome, which usually affects adult humans. The initial 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 may be due to chronic alcohol abuse, for example. Other common associations include eating disorders, bariatric surgery, malnutrition, chronic bowel disease with diarrhea and vomiting, or chemotherapy. Vitamin B1 deficiency, also known as thiamine deficiency, presents with hypovitaminosis. Thiamine is essential as a cofactor for intermediary metabolism, for example for processes such as ketoglutarate dehydrogenase, transketolase or pyruvate dehydrogenase. Intermediate metabolism is thus severely impaired by vitamin B1 deficiency. Energy metabolism experiences damage and cells perish. Because of the cell death, Wernicke’s encephalopathy is to be understood as a neurodegenerative disease and primarily affects brain areas with a high thiamine requirement. A particularly high demand is present in the corpora mamillaria, but the area surrounding the third ventricle of the brain, the nuclei of the thalamus, the corpora geniculata, or the aqueductal area may also be affected.
Symptoms, complaints, and signs
In the acute course of Wernicke’s encephalopathy, a reddish-brown discoloration can be seen macroscopically in brain regions in need of thiamine. Multiple petechial hemorrhages can be seen. Chronic progression results in atrophy of the corpora mamillaria. The histological picture is characterized by ganglion cell loss. Microscopically, spongy nerve disruption may occur, characterized by glial and vascular proliferations with siderophages. From a clinical standpoint, there is a classic triad of impaired consciousness or disorientation, gait ataxia, and ocular muscle dysfunction. In most cases, there is a brain-organic psychosyndrome characterized by cognitive dysfunction. Intellectual decline with memory loss may also be a characteristic symptom. In addition to ocular muscle paresis, nystagmus of the eyes may also be present. Additional symptoms may include polyneuropathies, reflex disorders, and dysdiadochokinesia. Equally common are dysphagia, dysarthria, or autonomic disorders such as hypotonia, hypothermia, or hyperhidrosis. Which disorders are present in detail depends on the individual case and the affected brain regions. Also, the respective cause the clinical picture in each case may vary more or less.
Diagnosis and course of the disease
The suspected diagnosis of Wernicke syndrome presents itself to the physician with the patient’s history and first impression of the clinical picture. In the medical history, 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 may yield false-negative values. Therefore, a more sensitive [[[whole blood test]]] is the diagnostic of choice. To further determine the location of brain lesions, imaging is usually used. Both CT and MRI are suitable procedures.The course of the disease depends on the primary cause of the vitamin deficiency. For example, a chronic bowel disease usually has a less favorable course than a currently acute bowel disease with only intermittent diarrhea and vomiting. In the case of Wernicke’s encephalopathy following alcohol abuse or eating disorders, the course depends exclusively on the patient’s cooperation. If left untreated, the disease can be fatal.
Complications
Wernicke’s encephalopathy has a very negative effect on the patient’s consciousness and can thus lead to various serious complaints. As a rule, affected individuals suffer from impaired consciousness and are unable to manage their daily lives on their own. There are disturbances in concentration and also in coordination, so that the patients are usually always dependent on the help of other people. This also prevents them from taking food and liquids. Often there is a loss of memory and furthermore various cognitive disorders, which have a negative impact on the patient’s quality of life. Most sufferers also lose consciousness of fall into a coma. The severity of the symptoms of Wernicke’s encephalopathy depends to a large extent on the exact cause, so that no general prediction can be made. However, 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. Frequently, the patient’s relatives are also affected by psychological complaints and therefore require psychological treatment as well.
When should one go to the doctor?
Changes and abnormalities in brain activity should always be examined and clarified by a doctor. If there are disturbances in eating behavior, diarrhea or vomiting, health check-ups are advisable. Symptoms of deficiency, changes in mental or physical performance as well as disorientation must be examined as soon as possible. In case of disturbances of consciousness as well as loss of consciousness, an ambulance service must be alerted. An acute health-threatening situation exists in which intensive medical care is necessary. In the event of excessive alcohol consumption, a reduction in physical strength, and a persistent or increasing feeling of illness, a physician is required. If the affected person refuses to eat or is unable to stop consuming alcohol on his or her own responsibility, the help of a physician must be sought as soon as possible. If disturbances of speech, states of confusion or an involuntary twitching of the muscles become apparent, the affected person requires medical assistance. Since Wernicke’s encephalopathy is fatal if left untreated, a visit to the doctor should be made at the first signs of irregularity. If the affected person is no longer able to fulfill the obligations of everyday life, and if behavioral abnormalities and changes in personality become apparent, this is considered extremely worrying. A doctor is needed to clarify the cause.
Treatment and therapy
Wernicke’s encephalopathy is treated with different focus depending on the primary cause. For example, if alcohol abuse is the primary cause, absolute abstinence from alcohol is the treatment recommendation of choice. The physician must make the patient aware of the context of his or her illness and usually works together 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 of choice for eating disorders. To mitigate Wernicke’s encephalopathy as a symptom of eating disorders or alcohol addiction in an acute phase, parenteral administration of thiamine in high doses is used. Absorption of oral thiamine is variable and poorly controlled. Therefore, intravenous administration is the more appropriate treatment route in emergency situations. Usually, about 200 milligrams of thiamine is administered over two days. However, a thrice-daily administration of 500 milligrams over two days is also an option. After completion of these measures, long-term oral administration over a certain period of time is recommended. In addition, the administration of magnesium is often added to the drug therapy.Chronic bowel disease patients usually require lifelong substitution of thiamine. For patients with malnutrition symptoms, an educational nutrition plan is also ideally provided. Nutritional plans may be generally useful in the context of therapy for Wernicke’s encephalopathies.
Prevention
Wernicke’s encephalopathy can be prevented to some extent via a balanced diet and responsible use of alcohol. However, as a sequelae of various bowel diseases, the disease cannot be prevented by these measures in all cases.
Aftercare
Follow-up treatment of Wernicke’s encephalopathy depends on the underlying disease. Because all cases of Wernicke’s encephalopathy are deficient in vitamin B-1, recurrence of vitamin B-1 deficiency must be prevented. For this purpose, vitamin B-1 should be taken as a precautionary measure in moderate doses as a dietary supplement in addition to the 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 alcoholism is responsible for the development of vitamin B-1 deficiency and the subsequent Wernicke’s encephalopathy, alcohol withdrawal must take place. This may require an inpatient stay in a detoxification clinic. If chemotherapy has led to the development of the disease, increased vitamin B-1 intake must occur. Since dietary supplements are often insufficient for this purpose during chemotherapy, this can also be done intravenously via infusions. If the cause of the vitamin B-1 deficiency underlying the disease cannot be clearly identified, various gastroenterological (colonoscopy) and endocrinological examinations (hormone tests) must be performed to determine and treat it. In addition, regular neurologic examinations of motor function, sensory function, reflexes, and coordination should be performed, as well as imaging of the brain (MRI, CT) to detect and treat long-term damage to the nerves and brain at an early stage.
Here’s what you can do yourself
The options for self-help are very limited in the event of an onset of Wernicke’s encephalopathy. There is a pre-existing condition that needs to be treated and therapized. Most often, it is an addictive disorder or a health disorder with a chronic course. The affected person should clarify and remedy the causes of the existing underlying disease to the best of his or her ability. This is only possible with difficulty, since he is usually already in an advanced stage of the present disease. Nevertheless, the consumption of harmful substances should be minimized under all circumstances. If possible, the intake of alcohol should be completely refrained from. Addicts only manage this step on their own in very few cases. Therefore, cooperation with a medical doctor as well as a psychotherapist is advisable. Motivation to change is the basic prerequisite for an improvement in the overall situation. Equally important is the strengthening of general well-being and the setting of life goals to be achieved. This approach supports behavioral changes and contributes significantly to alleviating existing symptoms. Targeted training and exercise sessions help to improve concentration. These can be implemented independently at any time and help with memory. In addition, a healthy and balanced diet is important for supporting the functioning of the organism.