Korsakoff syndrome
is a chronic memory disorder caused by severe deficiency of thiamine (vitamin
B-1). The most common cause is alcohol misuse.
Main Causes
Thiamine (vitamin B-1) helps brain cells produce energy
from sugar. When levels fall too low, brain cells cannot generate enough energy
to function properly. As a result, Korsakoff syndrome may develop.
Wernicke-Korsakoff syndrome (WKS) is a type of brain
disorder caused by a lack of vitamin B-1. The syndrome is actually two separate
conditions that can occur at the same time. Usually, people get the symptoms of
Wernicke’s encephalopathy first.
Also called Wernicke’s disease, people with Wernicke’s
encephalopathy have bleeding in the lower sections of the brain, including the
thalamus and hypothalamus. These areas of the brain control the nervous and
endocrine systems. The bleeding causes brain damage that presents symptoms
involving your vision, coordination, and balance.
The signs of Korsakoff psychosis tend to follow as the
Wernicke’s symptoms decrease. If Wernicke’s disease is treated quickly and
effectively, Korsakoff syndrome may not develop. Korsakoff psychosis is the
result of chronic brain damage. Korsakoff syndrome affects the areas of your
brain that control memory.
Lack of vitamin B1 is common in people who have alcohol
use disorder. It is also common in people whose bodies do not absorb food
properly (malabsorption). This can sometimes occur with a chronic illness or
after weight-loss (bariatric) surgery.
So, the number one cause of WKS is alcoholism. The less
common causes of WKS are conditions that limit nutritional absorption. Eating
and nutrient absorption can be restricted by:
* gastric bypass surgery, which makes it difficult to
meet nutritional needs due to limited food portions
* colon cancer, which can cause pain that causes you to
put off eating
* eating disorders
Symptoms
Symptoms of Wernicke encephalopathy include:
* Confusion and loss of mental activity that can progress
to coma and death
* Loss of muscle coordination (ataxia) that can cause leg
tremor
* Vision changes such as abnormal eye movements (back and
forth movements called nystagmus), double vision, eyelid drooping
* Alcohol withdrawal
Symptoms of Korsakoff syndrome:
* Inability to form new memories
* Loss of memory, can be severe
* Making up stories (confabulation)
* Seeing or hearing things that are not really there
(hallucinations)
Affected
Populations
Wernicke-Korsakoff syndrome occurs in 1-2 percent of the
general population in the United States. The disorder affects slightly more
males than females and is evenly distributed between ages 30-70.
Diagnosing WKS
Diagnosing WKS isn’t always easy. In the acute phase, a
physical examination may reveal skin changes and a red “beefy” tongue. In
addition, blood count, electrolytes, and liver function tests should be
conducted. Even in the chronic phase, an MRI may show shrunken mammillary
bodies and other changes in the brain. CT scans have showed enlarged ventricles
and diencephalic lesions.
An individual with WKS is often mentally confused. This
can make communication with the doctor difficult. Thus, the doctor may overlook
the possibility of a physical disorder if the patient is confused.
At the very first step of the medical investigation, the
doctor may first check for signs of alcoholism. It is important that a full
medical history include information about the person’s daily drinking habits,
both present and past. Family, friends, and past medical records should be
consulted to obtain the most complete information possible on the person’s
history with alcohol. Proposed criteria for diagnosing alcohol-related dementia
(not strictly WKS) suggest that the diagnosis be made at least 60 days after
the last exposure to alcohol and that a “significant” alcohol history would
include an average of 35 drinks per week for men (28 for women) for at least
five years. Typically, the period of significant drinking must be within three
years of the onset of dementia.
Recent medical research also suggests that the genetic
marker APOE4 is a significant predictor of global intellectual deficits in
people with WKS. Individuals with the APOE genotype may experience a certain
interaction with heavy alcohol use that could predispose them to WKS. Concerns
about an inherited susceptibility to WKS should be discussed with a genetic
counselor.
The doctor may also order nutritional tests to make sure the
patient isn’t malnourished. Nutritional tests may include the following:
·
A serum albumin test measures the levels of
albumin, which is a protein in the blood. Low levels of albumin may signal
nutritional deficiencies as well as kidney or liver problems.
·
A serum vitamin B-1 test is a blood test to
check vitamin B-1 levels in the blood. Enzyme activity in the red blood cells
can be tested. Low enzyme activity in the red blood cells signals a vitamin B-1
deficiency.
The diagnosing process might also include imaging tests.
These tests can help the doctor find any damage that’s characteristic of WKS.
Diagnostic imaging tests for WKS include:
·
an electrocardiogram (EKG) before and after
giving vitamin B-1, which can help your doctor find abnormalities
·
a CT scan to check for brain lesions related to
WD
·
an MRI scan to look for brain changes related to
WD
The doctor may also use neuropsychological test to
determine the severity of any mental deficiencies.
Treatment
Some experts recommend that heavy drinkers and others at
risk of thiamine deficiency take oral supplements of thiamine and other
vitamins under their doctor's supervision.
Many experts also recommend that anyone with a history of
heavy alcohol use who experiences symptoms associated with Wernicke
encephalopathy be given injectable thiamine until the clinical picture grows
clearer.
Once acute symptoms improve, individuals should be
carefully evaluated to determine if their medical history, alcohol use and
pattern of memory problems may be consistent with Korsakoff syndrome. For those
who develop Korsakoff syndrome, extended treatment with oral thiamine, other
vitamins and magnesium may increase chances of symptom improvement. Giving up
alcohol is also an effective treatment.
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.
The goals of treatment are to control symptoms and to
prevent the disorder from getting worse. Some people may need to stay in the
hospital early in the condition to help control symptoms.
Monitoring and special care may be needed if the person
is:
* In a coma
* Lethargic
* Unconscious
Vitamin B1 may be given by injection into a vein or a
muscle, or by mouth. This may improve symptoms of:
* Confusion or delirium
* Difficulties with vision and eye movement
* Lack of muscle coordination
Vitamin B1 often does not improve loss of memory and
intellect that occur with Korsakoff psychosis.
Stopping alcohol use can prevent more loss of brain
function and damage to nerves. A well-balanced, nourishing diet can help, but
it is not a substitute for stopping alcohol use.
Outlook
(Prognosis)
Without treatment, Wernicke-Korsakoff syndrome gets
steadily worse, and can be life threatening. With treatment, it is possible to
control symptoms (such as uncoordinated movement and vision difficulties). This
disorder can also be slowed or stopped.
Possible Complications
Complications that may result include:
* Alcohol withdrawal
* Difficulty with personal or social interaction
* Injury caused by falls
* Permanent alcoholic neuropathy
* Permanent loss of thinking skills
* Permanent loss of memory
* Shortened life span
Caring for the
Person with WKS
It is important to ensure that the affected person
continues to abstain from drinking alcohol, and that the person maintains a
balanced diet with adequate thiamine intake. However, even if the person stops
drinking and replenishes thiamine, symptoms of the disease (e.g., problem
behaviors, agitation, lack of coordination, learning deficits) may continue. In
an abstinent (i.e., sober) WKS patient, these symptoms must be recognized as
part of the disease caused by irreversible damage to the brain and nervous
system.
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, leave the stove on or
the water running.
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.
First, 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. In addition, place reminders in the house to help the person feel
more secure. Label inside doors and drawers with words or pictures. Write notes
(e.g., dinner is at 6:00 pm). Another strategy is to 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, symptoms should
be discussed with a physician, neurologist, or psychiatrist. Medications may be
available to help control outbursts or anxiety.
Just as important, it is essential that the caregiver get
some support and time off from constant caregiving demands. Make sure you leave
some time to attend to your own needs, including eating well, getting enough
sleep, and getting regular medical check-ups. A home care worker, friend, or
family member may be needed to provide periodic respite assistance to help your
loved one and to relieve the stress on you, the family caregiver.
Sources and
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