What is Wernicke Encephalopathy?
Wernicke encephalopathy (WE) is a serious and urgent brain disorder that typically exhibits three main symptoms: difficulty controlling eye movements, clumsy and unsteady movements, and confusion. This disease is caused by a lack of thiamine (a type of vitamin), which greatly affects both the peripheral and central nervous systems. It’s crucial to understand that this condition is different from Korsakoff syndrome, which can be prevented and is often seen after at least one episode of Wernicke’s encephalopathy.
Korsakoff syndrome is a brain disorder characterized by severe memory problems. Affected individuals may have difficulty forming new memories or recalling past events, while their immediate memory remains unaffected. Interestingly, despite these memory issues, their general awareness and understanding of their environment, called sensorium, remains intact. This condition can also lead some patients to make up stories, a behavior known as confabulating – often observed in both acute Wernicke state and chronic Korsakoff syndrome.
What Causes Wernicke Encephalopathy?
Thiamine deficiency, or lack of vitamin B1, is mostly associated with heavy alcohol use. While it’s common in people who drink a lot of alcohol, thiamine deficiency can also be caused by severe malnutrition, extreme morning sickness in pregnancy (known as hyperemesis gravidarum), long periods on fluid nutrition administered through a vein, cancers, diseases that weaken the immune system, liver disease, overactive thyroid, and severe eating disorders like anorexia nervosa. Drinking alcohol regularly can lead to lack of thiamine because it interferes with how the body absorbs thiamine from food, with genetic factors also playing a part. Inadequate diet, poor storage of thiamine in the liver, and other nutritional deficiencies can also contribute.
Often, Wernicke encephalopathy, a serious condition caused by lack of thiamine, can start suddenly due to an acute infection. Other triggers include consuming a large number of carbohydrates or sugars when there is a lack of thiamine in the body. In general, if you need to receive glucose, it’s recommended to take thiamine at the same time.
Risk Factors and Frequency for Wernicke Encephalopathy
Wernicke encephalopathy is a medical condition found in about 1% to 3% of autopsy studies. However, when looking at clinical records, it’s diagnosed less frequently, indicating it’s often missed or overlooked. Developing countries seem to have higher rates of this condition due to vitamin deficiencies and malnutrition. The condition affects both genders but is slightly more common in males with a ratio of 1 female to 1.7 males. There is no known racial group that is more prone to developing Wernicke encephalopathy.
Signs and Symptoms of Wernicke Encephalopathy
Wernicke encephalopathy (WE), a serious neurological condition, is often suspected in patients who have a history of chronic alcohol abuse or malnutrition. The usual signs of this disease include changes in mental status, eye movement problems, unsteady gait, delirium, and low blood pressure. However, it is important to note that the classic trio of symptoms (altered mental status, unsteady walk, and eye movement problems) may not be present in many patients, making its diagnosis based on these symptoms challenging.
One of the main features of WE is ocular abnormalities, particularly uncontrolled eye movements (nystagmus). The disease can also cause issues related to other eye muscles, leading to abnormalities in eye coordination or even droopy eyelids. Another significant symptom is an unsteady or broad-based walk, which can deteriorate to the point where the patient may not even be able to walk. Furthermore, WE may also lead to episodes of confusion and disorientation. In severe instances, it can cause a depressed level of consciousness, leading to coma and even death. Other symptoms could include a fever and low blood pressure. It may also cause sensory loss in the lower limbs due to peripheral neuropathy.
Long-term malnutrition and instances of confusing mental status could be linked to Wernicke encephalopathy. Also, there’s evidence to show that bariatric surgery, a procedure performed to help individuals lose weight, could be associated with WE. This is because post-surgery, individuals might intake less food, and key nutrients, like thiamine, get used up quickly, causing malnutrition.
Testing for Wernicke Encephalopathy
If your doctor suspects that you may have Wernicke’s encephalopathy, they’ll ask you about your medical history, complete a physical examination, and order some routine laboratory tests. They may also recommend that you have an image of your brain taken. You should know that there is no specific blood test to determine if you have Wernicke’s encephalopathy. Instead, doctors generally make a clinical diagnosis based on evidence from your medical history and physical examination.
For instance, your doctor may diagnose you with Wernicke’s encephalopathy if you have eye issues, signs of a B1 vitamin (thiamine) deficiency, an altered mental state, or problems with coordination and balance (cerebellar dysfunction).
Even though basic blood tests or imaging of the brain won’t provide a clear-cut diagnosis, they can help rule out other conditions that might be causing your symptoms. Certain brain scans might show distinctive patterns in people with Wernicke’s encephalopathy, like unusually bright signals in specific parts of the brain.
The diagnosis criteria established in 1997 by Caine and his colleagues are currently widely used. They state that if a patient has two or more symptoms like difficulty walking (ataxia), confusion or eye muscle paralysis (ophthalmoplegia), there is an 85% chance they have Wernicke’s encephalopathy.
Your doctor will also consider any existing risk factors you may have. While traditionally, Wernicke’s encephalopathy was most often seen in patients struggling with alcoholism, it can also occur in people who are chronically malnourished, have had weight loss surgery, severe morning sickness during pregnancy (hyperemesis gravidarum), liver diseases, overactive thyroid (hyperthyroidism), and severe anorexia nervosa.
Additionally, testing levels of specific substances in your red blood cells, such as transketolase, can hint at a thiamine deficiency. Your doctor might also check the levels of lactate and pyruvate – these are substances that need thiamine to be properly processed in your body.
Treatment Options for Wernicke Encephalopathy
The main goal of treatment is to quickly correct the shortage of thiamine (a type of vitamin) in the brain. Wernicke encephalopathy, a serious brain disorder, is a medical emergency. Even though the symptoms of this disorder can develop suddenly or over time, it’s crucial to treat it as soon as possible since it’s usually reversible with treatment. This treatment is typically delivered by injection to ensure a quick and effective response. However, persistent neurological problems may occur in some cases, and the condition can sometimes progress to a chronic state known as Korsakoff syndrome.
Thiamine treatment ideally should be given to the patient as quickly as possible. All undernourished patients may need higher doses of thiamine. Taking the thiamine orally is not as effective and is generally not recommended.
Treatment with thiamine can help improve symptoms such as confusion, unsteady walking, eye movement abnormalities, and rapid involuntary eye movements. Thiamine is typically given before or at the same time as glucose solutions. This is because the processing of glucose in the body can actually lower thiamine levels, making symptoms of Wernicke encephalopathy worse. If a patient also has a magnesium deficiency, they should be treated for this as well, particularly if they struggle with alcoholism. Treatment of both deficiencies is crucial for recovery.
Most patients will need to be hospitalized to ensure they receive thiamine and magnesium via an IV (a tube that goes into the vein).
What else can Wernicke Encephalopathy be?
When trying to figure out what’s going on with a patient, doctors might consider a few different conditions, including:
- Hepatic encephalopathy (a condition caused by serious liver problems)
- Stroke
- Alcohol withdrawal syndrome
- Delirium tremens (severe alcohol withdrawal)
- Chronic hypoxia (long-term lack of oxygen)
- Normal pressure hydrocephalus (a condition where fluid builds up in the brain)
What to expect with Wernicke Encephalopathy
Wernicke encephalopathy (WE) is a severe health condition that can pose a significant threat to life and personal wellbeing. The treatment with thiamine, a type of vitamin B, can lead to some improvement, but often the damage to a person’s cognitive abilities, such as thinking and memory, may still persist.
The confusion that typically accompanies WE usually gets better with thiamine given through an IV (a treatment where medicine is directly given into a vein). However, the issues with learning and memory only improve partially. In some rare cases, patients may not see any improvement and could develop a severe condition called Korsakoff psychosis, which may necessitate care in a specialized facility. Unfortunately, very few individuals recover once they reach this stage.
Possible Complications When Diagnosed with Wernicke Encephalopathy
Possible Health Problems:
- Damage to the nervous system
- Ataxia, a condition causing difficulty with movement and balance
- Korsakoff syndrome, a memory disorder often related to alcohol abuse
- Ophthalmoplegia, which is paralysis or weakness in the eye muscles
- Heart failure, where the heart is unable to pump blood effectively
- Lactic acidosis, a build-up of lactic acid in the body that can cause muscle pain and breathing difficulties
