Table of Contents
- What is It?
- What causes WKS, and what increases risk?
- Does my family member have it?
- How is WKS diagnosed?
- Important diagnostic criteria
- What’s the prognosis?
- How is WKS treated?
- What can I do to help our family member with WKS?
- How might this diagnosis affect our family?
- This is overwhelming. Is help available? Where do we start?
What is it?
Wernicke-Korsakoff syndrome (WKS) is a combination of two neurological conditions often blended together. Wernicke’s encephalopathy is the initial, acute stage of the syndrome. If the individual recovers from the acute stage, Korsakoff amnesic syndrome, the chronic, long-term stage of WKS results.
While there are many symptoms, the most significant feature of the syndrome is its effect on memory. Specifically, the condition causes difficulty in creating new memories or retrieving recent memories (memories made prior to the illness often remain intact).
WKS is less common than other kinds of dementia. It affects 1 to 2 percent of the U.S. population, but the disease is likely underreported, especially the milder forms which are difficult to recognize. Slightly more men than women are diagnosed. And it most often affects people between the age of 30 and 70.
What causes WKS, and what increases risk?
A severe deficiency in vitamin B1, thiamine, is the cause of WKS. Thiamine is a vital nutrient that helps metabolize sugar, which produces energy for the brain. Lack of thiamine kills brain cells and therefore causes neurological changes and degrees of cognitive impairment.
WKS is typically associated with chronic alcoholism. Alcohol use inhibits thiamine absorption, and heavy drinkers can have poor dietary habits. Medical researchers are still examining the association between heavy alcohol use and symptoms of dementia. There may be a hereditary aspect to WKS, as well. But the most common presentation of WKS is a chronic alcoholic patient who is poorly nourished.
Malnutrition and other causes of nutritional deficiencies can cause or worsen WKS. These include eating disorders, cancer, chemotherapy, gastric bypass, and kidney dialysisall conditions that deplete thiamine from the body. Inability to afford proper food and medical care also contribute to WKS.
Does my family member have it?
Some symptoms that family members are more likely to take note of can be subtle. Watch for changes, especially these:
- Mental confusion
- Vision problems
- Muscle weakness
- Muscle coordination issues, especially walking/gait
- Recent or short-term memory difficulties
- Exaggerated storytelling
Other, more acute symptoms that may indicate the need for more immediate medical attention include rapid heartbeat, low blood pressure, low body temperature, hallucinations, delirium, or stupor.
If you’ve seen any of the above signs or are concerned by other behavior, schedule an appointment with your family member’s primary care physician or, if needed, obtain immediate medical attention. And wherever the appointment takes place–in person, over the phone, or by video conference–be sure your family member has an advocate with them.
An advocate is someone who is there to support the patient, ask questions, and take notes of what is discussed. In this sort of situation, it will be impossible for a patient with serious memory problems to later share with family what happens during an appointment – so an advocate is vital.
Whether that advocate is you or another involved person, they should report back to family members about how the appointment went and communicate next steps – such as follow-up appointments, lab work, imaging tests, or home care advice.
How is WKS diagnosed?
Physicians diagnose WKS after a thorough clinical examination, including lab work, imaging scans, and a detailed review of the patient’s health record. The work-up commonly covers the following:
- A full medical history, including information about the person’s daily drinking habits—both past and present—and their dietary/nutritional history
- A physical examination to check for signs of malnutrition.
- Lab work that includes blood count, electrolytes, and liver function tests. Lab test results can reflect alcoholism-associated malnutrition or determine that the level of thiamine in the blood is below normal.
- An MRI or CT scan that may show enlarged ventricles and diencephalic lesions, shrunken mammillary bodies, and other changes in the brain.
- A referral by the psychiatrist or neurologist to a neuropsychologist is often necessary to quantify the degree of memory and other cognitive dysfunction and clarify the diagnosis.
Physicians should consult family, friends, and past medical records to get the most complete information possible on the person’s history with alcohol.
Important diagnostic criteria
These are the guidelines for diagnosing cases of alcohol-related dementia (not strictly WKS):
- A “significant” alcohol history, which includes an average of 35 drinks per week for men (28 for women), typically for 5 to 10 years or more.
- In order to obtain a reasonable diagnosis, 30 to 60 days should have transpired since the last exposure to alcohol. The longer without drinking and with good nutrition, the more accurate the diagnosis.
If physicians suspect non-alcohol related WKS, they may investigate other causes:
- anorexia nervosa
- hyperemesis gravidarum (an extreme form of morning sickness during pregnancy)
- severe malnutrition
- use of a feeding tube for an extended period of time without the addition of thiamine
- other disorders or surgical procedures that impair intestinal absorption of thiamine
WKS can often be missed as a diagnosis. You may find it helpful to bring this fact sheet with you to the appointment to help in talking about your concerns with the physician.
What’s the prognosis?
With a WKS diagnosis, treatment is critical and time sensitive.
If Wernicke’s encephalopathy is diagnosed and treated early, some or even all symptoms can be reversed. Once the chronic stage has set in recovery is less certain. Stopping alcohol use and ensuring a nutritious diet may prevent additional brain and nerve damage. Memory function, however, may be slow to improve; recovery is usually incomplete.
In the later stages, if damage to the brain is irreversible, individuals are likely to have lasting problems with memory and gait (for example, lack of muscle coordination and numbness or weakness in limbs).
Without treatment, according to the Alzheimer’s Association, the statistics can be discouraging:
- Wernicke’s encephalopathy can be incapacitating and life-threatening. It causes death in up to 20 percent of patients.
- In 85 percent of survivors, it progresses to Korsakoff syndrome.
- If the patient continues to use alcohol and follow a poor diet the condition and symptoms will worsen.
How is WKS treated?
- Thiamine replacement therapy administered as soon as possible.
- Consistent, good hydration and nutrition.
- Abstaining from alcohol—this is key.
Research is being conducted on WKS.
- Clinical trials of the National Institute of Neurological Disorders and Stroke, a division of the National Institutes of Health: https://www.ninds.nih.gov/Disorders/Clinical-Trials/Find-Ninds-Clinical-Trials.
- Publicly and privately funded clinical studies around the world, from the NIH’s U.S. National Library of Medicine’s database, ClinicalTrials.gov: https://clinicaltrials.gov/search/term=Wernicke-Korsakoff%20Syndrome.
What can I do to help our family member with WKS?
- It is important to ensure that the affected person continues to abstain from drinking alcohol.
- The person must maintain a nutritious, balanced diet and stay properly hydrated.
- In the short term, it is critical that your family member have adequate, physician-recommended thiamine intake. In the long term, if your family member has a balanced diet and is well nourished, thiamine supplementation may not be necessary—check with your doctor.
Keep reasonable, realistic expectations
Even if the affected person stops drinking and replenishes thiamine, they may continue to show signs of the syndrome (e.g., confusion, lack of coordination, trouble with memory and recall). In a sober WKS patient, it’s important to recognize these symptoms as part of the disease caused by irreversible damage to the brain and nervous system.
Remember that in some sober WKS patients, those who do not know them might consider them unimpaired, as they can carry on an intelligent and entertaining discussion which belies their ability to carry on with life independently.
Family caregivers should take precautions to ensure the safety of the person with WKS, as well as others in the household.
- The confused or disoriented individual should not be left alone.
- Supervision is required to ensure that the person does not wander away from home or leave the stove on or the water running.
Responding to memory lapses, reacting to repetition
Short-term memory problems mean that the confused person may repeat the same question again and again. Coping with frequent repetition often involves a trial-and-error approach and a combination of strategies.
- Be patient and deliver responses in a calm manner. The confused person will pick up on your mood and may become more frustrated if your voice is loud or angry.
- Place reminders in the house to help the person feel more secure in their environment. Label inside doors and drawers with words or pictures of what’s inside. Write notes (e.g., dinner is at 6:00 pm).
- Distract the person with another topic or activity (e.g., a short walk, reminiscing over an old photo, etc.).
If the person continues to be agitated, discuss the symptoms with a physician, neurologist, or psychiatrist. Medications may be available to help control outbursts or anxiety.
Caring for the caregiver
Just as important, the caregiver needs support and time off from constant caregiving demands. Make sure you leave time to attend to your own.
- Start with fundamental acts of self care, including eating well, getting enough sleep, and getting regular medical check-ups.
- Plan a schedule of periodic respites for yourself. You might need a home care worker, friend, or family member to help your loved one to provide you, the family caregiver, with a respite to relieve stress.
How might this diagnosis affect our family?
Caring for a family member who has WKS or alcohol-related dementia presents challenges for family caregivers. Lasting symptoms of dementia and other neurological problems are difficult conditions under even the best circumstances. Family members may interpret the affected person’s bizarre or confusing behaviors as a continuation of “binge” drinking, even if the person has stopped drinking.
Alcoholism and family dynamics
Strained relationships are common in families of alcoholics.
- Individuals with a history of alcohol abuse have often isolated themselves from their families and loved ones.
- As a caregiver, you may feel resentful of caring for a parent or spouse with a life-long history of alcohol abuse.
- Most WKS-affected individuals have been long-term alcoholics, so It may be hard to convince your family member to give up drinking. Talk with a physician or mental health professional about effective strategies for prevention and management.
- Research indicates that alcoholism often runs in families. This can put even more stress on family relationships.
- Having additional family members who are alcoholic increases the burden of care.
- Research has shown that individuals may inherit a susceptibility to alcohol addiction and alcohol-related neurological problems (peripheral neuropathies).
- In alcoholic families, family members should take special precautions to avoid excessive use of alcohol to reduce their own risk of alcohol-related health problems.
Professionals can help
Mental health professionals and case workers experienced in addiction can offer much-needed help and support.
- A case manager or counselor can help the family sort through issues and help arrange appropriate support services.
- Counselors and case workers can also help with family meetings and may be helpful in bringing together family members to assist the WKS person. They can also recommend support groups.
- In severe cases, or when the family is unable to provide appropriate care, you may want to look into a residential facility. If a WKS patient experiences persistent confusion, there are nursing homes that provide special dementia care.
This is overwhelming. Is help available? Where do we start?
Family Caregiver Alliance
National Center on Caregiving
(415) 434-3388 | (800) 445-8106
FCA CareNav: www.fca.cacrc.org/
Caregiver Services by State: https://www.caregiver.org/connecting-caregivers/services-by-state/
Family Caregiver Alliance (FCA) seeks to improve the quality of life for caregivers through education, services, research, and advocacy. Through its National Center on Caregiving, FCA offers information on current social, public policy, and caregiving issues and provides assistance in the development of public and private programs for caregivers. For residents of the greater San Francisco Bay Area, FCA provides direct family support services for caregivers of those with Alzheimer’s disease, stroke, ALS, head injury, Parkinson’s, and other debilitating brain disorders that strike adults.
Other Organizations and Links
National Organization for Rare Disorders
National Institute on Alcohol Abuse and Alcoholism
American Association for Marriage and Family Therapy
National Eldercare Locator
Caregiver Action Network
Al-Anon Family Groups
This information was prepared by Family Caregiver Alliance, updated by Patricia Doherty, and reviewed by Peter R. Martin, MD, MSc, DABPN, DLFAPA, FAAAP, Professor of Psychiatry and Behavioral Sciences, Professor of Pharmacology, Vanderbilt Psychiatric Hospital. Sources for this information include the National Organization for Rare Disorders (https://www.rarediseases.org), the NIH’s National Institute of Neurological Disorders and Stroke (https://www.ninds.nih.gov), Alzheimer’s Association (https://www.alz.org), ©2021 Family Caregiver Alliance. All rights reserved.