Series: What's Under the Dementia Umbrella?

Part 7: Wernicke-Korsakoff

In this series we are taking an in-depth look at the various types of dementia and how to improve treatment in a long-term care setting. There are several lesser known dementias affecting our seniors. As part of their care team we need to educate ourselves on how to better understand and meet their needs.

Today we will examine Wernicke-Korsakoff syndrome. Thanks to the Alzheimer’s Association, the National Institute of Neuroglogical Disorders and Stroke and Family Caregiver Alliance for providing the information for this article. Visit these organizations online for further information.

Overview of Wernicke-Korsakoff Syndrome

Wernicke-Korsakoff Syndrome, or WKS, is commonly split into Wernicke’s encephalopathy and Korsakoff syndrome (also known as Korsakoff's amnesic syndrome). Experts disagree as to whether these are related disorders or stages of one syndrome. If they are stages, Wernicke’s would be the acute phase while Korsakoff’s is the later, chronic stage.

Wernicke's encephalopathy, a degenerative brain disorder, results from a lack of thiamine (vitamin B1). Without enough thiamine, the brain doesn’t have enough energy to function and damage occurs in the thalamus and hypothalamus, causing mental confusion, coma, hypothermia, lack of muscle coordination (ataxia), problems with vision and low blood pressure. This deficiency of thiamine can arise from alcohol abuse, anorexia, overly-stringent dieting, fasting, starvation, weight-loss surgery, uncontrolled vomiting, AIDS, kidney dialysis, chronic infection or cancer that has spread throughout the body.

The inability of the brain to create enough energy can lead to damage of the nerve cells, supporting cells in the brain and spinal cord as well as the memory center of the brain. This is the marker of Korsakoff syndrome. This results in the patient having trouble acquiring new information, establishing new memories and recalling old memories. While symptoms can include amnesia, tremor, coma, disorientation and vision problems, thinking and social skills remain sharp even in the presence of crippling issues with memory. For example, a patient with Korsakoff syndrome may have a perfectly normal conversation with you and only moments later forget whom they spoke with or forget the conversation entirely. Patients with this syndrome are known to make up information they can’t remember. This is referred to as confabulating. It is important to understand that they aren’t being deceitful; people with Korsokoff syndrome may actually believe their own made up stories. Research has not yet uncovered the reason for confabulation.

Diagnosis

This most common cause of WKS is alcoholism. This makes diagnosis difficult because the symptoms of alcoholism, such as intoxication, head injuries sustained while intoxicated, withdrawal and infection, can mask the symptoms of WKS. In the acute phase, a patient may present with skin changes and a red, “beefy” tongue. Because the diagnosis of WKS is often missed, physical examination, blood count, electrolytes and liver function tests should be conducted. An MRI can show shrunken mammillary bodies and other changes in the brain in the chronic phase while CT scans have revealed enlarged ventricles and diencephalic lesions.

Prognosis

Both disorders can be disabling and life-threatening if they go untreated, but it’s possible to reverse a majority of the symptoms associated with Wernicke’s encephalopathy if prompt and thorough treatment follow detection. Cutting off the use of alcohol may prevent further damage to the brain. Memory function, however, is slow to improve and usually will not be completely regained.

The Alzheimer’s Association states, “In those who develop Korsakoff syndrome, with or without a preceding episode of Wernicke encephalopathy, there are few studies on long-term outcomes. Available data suggest that about 25 percent of those who develop Korsakoff syndrome eventually recover, about half improve but don't recover completely and about 25 percent remain unchanged. Some research suggests that those who recover from an episode may have a normal life expectancy if they abstain from alcohol.”

Treatment

In cases caused by alcoholism, the first step of treatment is stopping the use of alcohol, followed by replacing lost thiamine, proper nutrition, hydration and, in some cases, drug therapy. In the case of Wernicke's encephalopathy, thiamine replacement must take place prior to replenishing the body’s nutrition deficits. Vitamins and magnesium may also be effective treatments.

Caring for a Person with WKS

The ultimate goal in caring for a person with WKS is keeping them safe. Patients will often forget what they’re doing right in the middle of an activity. They might leave the water running or food cooking on the stove. Other symptoms will be similar to those observed in people with other dementias: problem behaviors, agitation, a lack of coordination and learning deficits.

People with WKS are often repetitious. They will ask the same questions or tell the same stories over and over. These stories, remember, may not be true as the patient confabulates. While a caregiver can easily become exhausted with repeating themselves, it’s important to stay calm and avoid arguing, even when you know what the patient is saying isn’t true. Remember, they’re not actually lying to you and will pick up on any frustration, further exacerbating their own agitation.

For patients who are no longer able to walk or feed themselves, caregivers often use assistive devises. Keeping to a schedule is helpful for a person with WKS, as is writing them notes. Redirection and distraction are effective tools to calm their mood.

For family members who are primary caregivers for someone with WKS, joining Alcoholics Anonymous can provide tremendous support as well as resources.