Tag Archive | Wernicke-Korsakoff Syndrome

Confabulation, Alcohol Abuse, and Alcohol-Related Dementia

Confabulation breaks trustOne of the lifelong struggles we, as human beings, do – or should be doing – battle with is being consistently honest both with ourselves (I submit this may be the hardest part of this battle because our capacity for self-deception seems to have no limits) and with others.

I have written here before about the interconnected relationship between honesty and trust. When we are dishonest with someone, we break their trust. Continue reading

The Layperson’s Guide to Alcohol-Related Dementia

pouring-shots-alcohol-related-dementia

In “Lifestyle Dementia: Underdiscussed, Overlooked, But a Very Real and Present Danger,” and “Is the Precipitous Rise in Dementias and Alzheimer’s Disease Over the Last Twenty to Thirty Years Linked to Lifestyle?,” we see that certain lifestyle factors and choices can make the likelihood of developing dementias and Alzheimer’s Disease more probable.

Two lifestyle factors that can contribute to the development of dementias and Alzheimer’s Disease – and the onset of these is usually before age 65 – are chronic, long-term alcohol abuse and alcoholism. This type of dementia is called alcohol-related dementia and can manifest itself in various forms.

This post will take a look at how chronic, long-term alcohol abuse and alcoholism affects the brain and what the behaviors and symptoms of the dementia looks like.

We all know that drinking enough alcohol at one time impairs the brain. Common symptoms include slurring words, exhibiting general motor impairment, including stumbling and walking off-balance, making poor decisions (like driving, for example), being less able to hear sound at a normal volume, experiencing vision problems, and being unable to think clearly. 

These behaviors occur because alcohol depresses the central nervous system , causing it to slow down its responses and reactions. The brain stem (made up of the Pons, Medulla, and Midbrain), which regulates breathing, heart rate, lifestyle dementia alcohol related going gentle into that good nightand consciousness, as well all other areas of the brain are affected by alcohol:

  • Frontal – involved in movement, problem-solving, concentrating, thinking, mood, behavior, and personality
  • Temporal – involved in hearing, language, and memory
  • Parietal – involved in sensation awareness, language, perception, attention, and body awareness 
  • Occipital – involved in vision and perception
  • Cerebellum – involved in posture, balance, and coordination of movement

Chronic, long-term alcohol abuse and alcoholism have even more devastating – and permanent – effects on the brain, eventually leading to alcohol-related dementia.

Usually the first noticeable symptoms of chronic, long-term alcohol abuse and alcoholism are cognitive. Memory loss is common, but a unique feature of memory loss with people who are chronic, long-term alcohol abusers or alcoholics is confabulation.

Confabulation occurs when, instead of recalling accurate memories because of the damage to the brain, the person distorts, makes up, and misinterprets memories about themselves, others, and the world around them.

As difficult as it is to believe for those on the receiving end of confabulation, there is no conscious intent to be dishonest. It is simply the result of extensive neurological damage.

One of the most challenging aspects of people who confabulate is that although they are giving blatantly false information, the information can appear to be coherent, internally consistent, and relatively normal.

People who confabulate have incorrect memories that run the gambit from slight, almost imperceptible changes to the most outlandish made-up stories you can imagine.

The maddening thing about this is that they generally very confident – to the point of arguing down anyone (because they know the memory is fabricated) who tries to correct or challenge them – about their recollections, despite overwhelming concrete evidence that contradicts them.

Other signs of alcohol-related dementia emerge as:

  1. Inappropriate behavior, including words and actions
  2. Loss of executive function, including organizing and planning
  3. Slowed thinking, reactions, and speaking
  4. Garbled speech
  5. Trouble executing basic skills functions like adding, subtracting, multiplying, and dividing
  6. Decreased ability to concentrate
  7. Decreased ability to complete tasks
  8. Trouble with balance
  9. Diminished hearing

With alcohol-related dementia, as with all other dementias, the person who has alcohol-related dementia loses the self-awareness that anything is wrong, both neurologically and behaviorally.

Most cases of alcohol-related dementia involve global neurological deterioration. Everything is affected.

However, two very specific types of alcohol-related dementia, Wernicke encephalopathy and Korsakoff syndrome (known together as Wernicke-Korsakoff Syndrome), which are the result of a vitamin B1 (thiamine) deficiency, have key features specific to them. There can be some reversal of symptoms with B1 (thiamine) therapy, but there is still permanent neurological damage and concurrent alcohol-related dementia.

Wernicke encephalopathy (commonly known as “wet brain”) causes damage in the thalamus and hypothalamus. Its symptoms include:

  • alcohol-related dementia Wernicke encephalopathy going gentle into that good nightSevere confusion and decreased mental activity that can lead to comas and death
  • Loss of muscle coordination (ataxia) that can cause tremors in the legs
  • Vision deterioration including abnormal eye movements, drooping eyelids, and persistent double vision

As symptoms of Wernicke encephalopathy disappear, Korsakoff syndrome symptoms appear. These include:

  • Loss of ability to form new memories
  • Moderate to severe loss of all memories
  • Confabulation
  • Visual and auditory hallucinations  

Malcolm Young, the 61-year-old co-founder and guitarist for the band AC/DC, has been moved to a nursing home and his family has confirmed that he has dementia (he’s unable to remember any of the band’s songs).

Young’s addiction to alcohol is well-known. Although he sought rehabilitation treatment for alcoholism during the band’s tour in 1988, it appears that he malcolm young ac/dc dementia going gentle into that good nightrelapsed (the statistics on the efficacy of alcohol rehab are grim: from 50 to 90% of people who’ve been through treatment relapse, often, over a period of time, habitually consuming even more alcohol than they did before entering treatment) and never sought treatment again.

In April of this year, Young was hospitalized with what was described to the media as a stroke (chronic alcohol abuse has very detrimental effects on blood, including causing the platelets to clump together and form clots, and these clots, when they travel to the brain are responsible for strokes), so this would be entirely consistent with what we know about Young’s lifestyle. 

There are systemic physiological effects of chronic, long-term alcohol abuse and alcoholism, including nerve damage in the arms and legs (peripheral neuropathy), liver damage (cirrhosis), heart damage, and kidney damage.

Concurrent with all of that is the irreversible neurological damage to the brain that results in alcohol-related dementia, which can emerge as early as 30 years of age, but more commonly begins emerging after the age of 50 in chronic, long-term alcohol abusers and alcoholics.

Drinking alcohol in moderation is fine. But I urge you to take an honest look at your drinking patterns and behavior. If you find that you are a chronic, long-term alcohol abuser or an alcoholic, then it’s time today to find a way to stop drinking alcohol for good.

But no one else can do that for you. Only you can make the choice to stop drinking alcohol and then follow through with actually doing it for the rest of your life. 

And here’s the key: until the rest of your life becomes more important than alcohol, you will be unsuccessful at choosing and taking action to stop drinking alcohol.

Because you are the only one who can take the action, every time you drink alcohol, as a chronic, long-term alcohol abuser or an alcoholic, you show yourself and the rest of the world the choice you’re making and you show yourself and the rest of the world what the most important thing in your life is.

And no one can change that but you.

Alcohol-Related Dementia: A Lifestyle Dementia

pouring-shots-alcohol-related-dementia

In “Lifestyle Dementia: Underdiscussed, Overlooked, But a Very Real and Present Danger,” and “Is the Precipitous Rise in Dementias and Alzheimer’s Disease Over the Last Twenty to Thirty Years Linked to Lifestyle?,” we see that certain lifestyle factors and choices can make the likelihood of developing dementias and Alzheimer’s Disease more probable.

Two lifestyle factors that can contribute to the development of dementias and Alzheimer’s Disease – and the onset of these is usually before age 65 – are chronic, long-term alcohol abuse and alcoholism. This type of dementia is called alcohol-related dementia and can manifest itself in various forms.

This post will take a look at how chronic, long-term alcohol abuse and alcoholism affects the brain and what the behaviors and symptoms of the dementia looks like.

We all know that drinking enough alcohol at one time impairs the brain. Common symptoms include slurring words, exhibiting general motor impairment, including stumbling and walking off-balance, making poor decisions (like driving, for example), being less able to hear sound at a normal volume, experiencing vision problems, and being unable to think clearly. 

These behaviors occur because alcohol depresses the central nervous system , causing it to slow down its responses and reactions. The brain stem (made up of the Pons, Medulla, and Midbrain), which regulates breathing, heart rate, lifestyle dementia alcohol related going gentle into that good nightand consciousness, as well all other areas of the brain are affected by alcohol:

  • Frontal – involved in movement, problem-solving, concentrating, thinking, mood, behavior, and personality
  • Temporal – involved in hearing, language, and memory
  • Parietal – involved in sensation awareness, language, perception, attention, and body awareness 
  • Occipital – involved in vision and perception
  • Cerebellum – involved in posture, balance, and coordination of movement

Chronic, long-term alcohol abuse and alcoholism have even more devastating – and permanent – effects on the brain, eventually leading to alcohol-related dementia.

Usually the first noticeable symptoms of chronic, long-term alcohol abuse and alcoholism are cognitive. Memory loss is common, but a unique feature of memory loss with people who are chronic, long-term alcohol abusers or alcoholics is confabulation.

Confabulation occurs when, instead of recalling accurate memories because of the damage to the brain, the person distorts, makes up, and misinterprets memories about themselves, others, and the world around them.

As difficult as it is to believe for those on the receiving end of confabulation, there is no conscious intent to be dishonest. It is simply the result of extensive neurological damage.

One of the most challenging aspects of people who confabulate is that although they are giving blatantly false information, the information can appear to be coherent, internally consistent, and relatively normal.

People who confabulate have incorrect memories that run the gambit from slight, almost imperceptible changes to the most outlandish made-up stories you can imagine.

The maddening thing about this is that they generally very confident – to the point of arguing down anyone (because they know the memory is fabricated) who tries to correct or challenge them – about their recollections, despite overwhelming concrete evidence that contradicts them.

Other signs of alcohol-related dementia emerge as:

  1. Inappropriate behavior, including words and actions
  2. Loss of executive function, including organizing and planning
  3. Slowed thinking, reactions, and speaking
  4. Garbled speech
  5. Trouble executing basic skills functions like adding, subtracting, multiplying, and dividing
  6. Decreased ability to concentrate
  7. Decreased ability to complete tasks
  8. Trouble with balance
  9. Diminished hearing

With alcohol-related dementia, as with all other dementias, the person who has alcohol-related dementia loses the self-awareness that anything is wrong, both neurologically and behaviorally.

Most cases of alcohol-related dementia involve global neurological deterioration. Everything is affected.

However, two very specific types of alcohol-related dementia, Wernicke encephalopathy and Korsakoff syndrome (known together as Wernicke-Korsakoff Syndrome), which are the result of a vitamin B1 (thiamine) deficiency, have key features specific to them. There can be some reversal of symptoms with B1 (thiamine) therapy, but there is still permanent neurological damage and concurrent alcohol-related dementia.

Wernicke encephalopathy (commonly known as “wet brain”) causes damage in the thalamus and hypothalamus. Its symptoms include:

  • alcohol-related dementia Wernicke encephalopathy going gentle into that good nightSevere confusion and decreased mental activity that can lead to comas and death
  • Loss of muscle coordination (ataxia) that can cause tremors in the legs
  • Vision deterioration including abnormal eye movements, drooping eyelids, and persistent double vision

As symptoms of Wernicke encephalopathy disappear, Korsakoff syndrome symptoms appear. These include:

  • Loss of ability to form new memories
  • Moderate to severe loss of all memories
  • Confabulation
  • Visual and auditory hallucinations  

Malcolm Young, the 61-year-old co-founder and guitarist for the band AC/DC, has been moved to a nursing home and his family has confirmed this week that he has dementia (he’s unable to remember any of the band’s songs).

Young’s addiction to alcohol is well-known. Although he sought rehabilitation treatment for alcoholism during the band’s tour in 1988, it appears that he malcolm young ac/dc dementia going gentle into that good nightrelapsed (the statistics on the efficacy of alcohol rehab are grim: from 50 to 90% of people who’ve been through treatment relapse, often, over a period of time, habitually consuming even more alcohol than they did before entering treatment) and never sought treatment again.

In April of this year, Young was hospitalized with what was described to the media as a stroke (chronic alcohol abuse has very detrimental effects on blood, including causing the platelets to clump together and form clots, and these clots, when they travel to the brain are responsible for strokes), so this would be entirely consistent with what we know about Young’s lifestyle. 

There are systemic physiological effects of chronic, long-term alcohol abuse and alcoholism, including nerve damage in the arms and legs (peripheral neuropathy), liver damage (cirrhosis), heart damage, and kidney damage.

Concurrent with all of that is the irreversible neurological damage to the brain that results in alcohol-related dementia, which can emerge as early as 30 years of age, but more commonly begins emerging after the age of 50 in chronic, long-term alcohol abusers and alcoholics.

Drinking alcohol in moderation is fine. But I urge you to take an honest look at your drinking patterns and behavior. If you find that you are a chronic, long-term alcohol abuser or an alcoholic, then it’s time today to find a way to stop drinking alcohol for good.

But no one else can do that for you. Only you can make the choice to stop drinking alcohol and then follow through with actually doing it for the rest of your life. 

And here’s the key: until the rest of your life becomes more important than alcohol, you will be unsuccessful at choosing and taking action to stop drinking alcohol.

Because you are the only one who can take the action, every time you drink alcohol, as a chronic, long-term alcohol abuser or an alcoholic, you show yourself and the rest of the world the choice you’re making and you show yourself and the rest of the world what the most important thing in your life is.

And no one can change that but you.

The Layperson’s Guide to Early-Onset Dementias

There are several types of common early-onset dementias. Early-onset dementias are categorized as dementias where the onset of symptoms is prior to age 65. These dementias can occur as early as the 30’s, but more typically become symptomatic in the 40’s and 50’s.

Early-onset dementias, unfortunately, are still off the main grid for medical staff – a classic instance of fixed expectations that dementias won’t be an issue for a person until after age 65 – and our loved who are diagnosed with early-on dementias face challenges that our older loved ones who are suffering with these diseases don’t face. These include:

  • Difficulty getting a correct diagnosis
  • Loss of employment and income
  • Difficulty getting Social Security Disability Insurance, Medicaid, and other employment-related disability insurance
  • Loss of health insurance and high-out-of-pocket costs for medical care
  • High out-of-pocket costs for long-term care
  • Lack of appropriate medical care, residential care, and community services (all of these are geared toward an older population)

Early-onset dementias typically are harder to diagnose because other than the dementia systems, sufferers are usually healthy, active, and aware there is a problem.

Additionally, the symptoms of early-onset dementias usually don’t have memory impairment as the predominant feature. Most often, behavioral and personality changes occur first, so usually the first type of treatment is psychiatric instead of neurological.

The causes of early onset-dementias fall into three categories: random, genetic, and lifestyle.

Random early-onset dementias are just that. There’s no concrete link to a cause. My opinion is that few of these in this category are actually random, but the causative issue(s) have not been identified yet.

Genetics plays an important role in certain early-onset dementias (and, although the scientific community has overlooked or disregarded the familial aspect of elder-onset dementias, it appears very likely, from observation, that if there’s a family history of elder-onset dementias, there may be a genetic predisposition for development of elder-onset dementias in subsequent generations).

Three genes are known to have mutations in the case of some sufferers of early-onset dementia, Alzheimer’s type (symptoms related to these genetic mutations usually begin in the 30’s and 40’s):

  • Amyloid precursor protein gene (APP) on chromosome 21
  • Presenillin-1 (PSEN-1) on chromosome 14
  • Presenillin-2 (PSEN-2) on chromosome 1 

We’ve talked extensively here about lifestyle dementias with regard to management of health (blood pressure and blood sugar) and substance abuse, as well as with regard to what we eat and how we live daily life. Some of the early-onset dementias we will talk about here can be directly attributed to lifestyle.

There are several types of early-onset dementias.

At least 1/3 of all sufferers diagnosis with early-onset dementia have Alzheimer’s Disease (remember that Alzheimer’s Disease is a type of dementia, but is not inclusive of all types of dementia, just as all photocopiers are not Xerox photocopiers and all facial tissues are not Kleenex facial tissues).

Onset symptoms include progressive and episodic memory loss, as well as visuospatial and perceptual deficiencies, but intact language and social functioning.

early-onset dementia, Alzheimer's type, Pat SummittThis type of early-onset dementia is more common in women than men. Once symptoms appear, the duration of the disease averages eight years.

A recent example is the 2011 diagnosis of former University of Tennessee women’s head basketball coach, Pat Summit, who was diagnosed with early-onset dementia, Alzheimer’s type, at age 59. Coach Summitt stayed with the team one more season, but was not actively coaching that season.

Coach Summitt retired in 2012 and has begun the Pat Summit Foundation to raise Alzheimer’s Disease awareness.

"Still Alice" by Lisa Genova - early-onset dementia, Alzheimer's typeThe novel, Still Alice, written by neuroscientist Lisa Genova, gives a scientific, compassionate and compelling look from the inside out of a 50-year-old Harvard psychology professor as early-onset dementia, Alzheimer’s type enters and progresses through her life.

Since the publication of Still Alice in 2007, Genova has continued her work with bringing the neuroscience of all types of dementias in the same compassionate and compelling style of her first novel through subsequent books and through documentaries produced with her husband, who is a filmmaker.

MRI-vascular-dementia-diffuse-white-matterThe second most common type of early-onset dementia is vascular dementia. Vascular dementia can occur because of:

  • Multiple cortical infarcts (small areas of tissue that have died from the lack a blood supply) that are most often caused by transient ischemic attacks (TIA’s) or silent strokes and characterized by stepwise deterioration of cognitive function
  • Small-vessel disease, resulting in a more subtle decline of cognitive function
  • Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
    • Rare cause of early-onset subcortical strokes and dementia
    • Caused by a mutation of Notch 3 gene on chromosome 19
    • MRI shows diffuse white matter lesions on the cerebral hemispheres, especially in the anterior temporal lobes and external capsules

With early-onset vascular dementia, there are usually lifestyle factors involved such as uncontrolled or undetected high blood pressure and an unhealthy diet. Recent scientific research has also linked high cholesterol levels with the development of vascular dementia.

frontal and temporal lobes function FTD early-onset dementiaThe third most common type of early-onset dementia is frontotemperal dementia (FTD), also known as Pick’s Disease, which affects the frontal and temporal lobes of the brain. FTD usually has an onset between the ages of 45 and 65. Its average duration is eight years. 

There are three types of FTD: behavioral variant FTD, semantic dementia, and primary progressive (also known as progressive nonfluent) aphasia.

In about half the cases of FTD, there is a positive family history for the disease, indicating a probable genetic link (although researchers have not yet identified the genetic mutation).

FTD can co-occur with motor neuron diseases (ALS, also known as Lou Gerhig’s Disease, is an example of a motor neuron disease), but only about 10% of sufferers of only motor neuron diseases develop dementia, resulting in a very aggressive course of the illness.

FTD presents differently from early-onset Alzheimer’s Disease and early-onset vascular dementia because the first symptoms involve changes in personality and social conduct while memory, perception, and visuospatial skills remain unchanged.

The most common indicators are:

  • Behavior disturbances
  • Personality changes
  • Decreased motivation
  • Reduced empathy
  • Impaired planning
  • Impaired judgment
  • Speech and language difficulties

As FTD progresses, other symptoms become apparent:

  • Difficulty behaving appropriately in new and unfamiliar situations
  • Loss in inhibitions (disrobing is not uncommon)
  • Loss of social skills
  • Emotional outbursts
  • Impulsivity
  • Executive function deficits
  • Decreased verbal fluency
  • Compulsive or repetitive behavior
  • Lack of insight
  • Self-neglect
  • Inappropriate sexual behavior

The semantic dementia form of FTD includes symptoms of:

  • Difficulty with correctly naming objects (people, places, and things)
  • Impaired understanding of the meaning of words
  • Inability to understand substitute words

However, in this form of FTD, speech remains fluent and cognition remains intact. MRI scans show more atrophy of the anterior temporal lobe than the posterior temporal lobe.

The primary progressive (progressive nonfluent) aphasia form of FTD is characterized by:

  • Progressive decline in all language skills, with no other cognitive deficits
  • Increased difficulty with speech and speaking (by the end of the disease, most sufferers don’t speak at all)
  • Speech and speaking is not fluent and requires a great deal of effort

MRI scans show predominant atrophy of the left perisylvian region of the temporal lobe.

The fourth most common type of early-onset dementia is Lewy Body dementia. I’ve included the link to my post about Lewy Body dementia for a full description, but will include a brief summary of the dementia’s Lewy Body Protein - Lewy Body dementiaprimary symptoms:

The fifth most common type of early-onset dementia is Wernicke-Korsakoff Syndrome (alcohol-related dementia). This is a lifestyle dementia, brought on by long-term, heavy alcohol consumption.

wernicke-korsakoff dementia (alcohol-related dementia)

Characteristics of Wernicke-Korsakoff Syndrome include:

  • Damage to the limbic structures and frontal lobes
  • Memory impairment
  • Executive functioning impairment
  • Autobiographical memory is frequently affected resulting in confabulation (making up stories)
  • Memory loss stops where it is when drinking stops (damage already done remains)

As shown by the MRI scan above, there is general cortical atrophy along with damage to the frontal, parietal and cingulated regions of the brain, with the majority of the damage occurring in the frontal lobe.

There are two other less common types of early-onset dementia that we’ll discuss.

One is Huntington’s Disease. As this genetically-inherited disease progresses, dementia develops.

MRI Huntington's DiseaseEveryone is born with this gene. However, in Huntington’s Disease, an inherited mutated copy of this gene (on chromosome 4), produces a defective form of the huntingtin protein that causes degeneration and death of the neurons, especially in the center of the brain. 

Because this gene is a dominant gene (as opposed to a recessive gene), everyone who inherits the mutated copy of the gene will, at some point, develop Huntington’s Disease.

Symptoms typically appear between ages 30 and 50, but it can begin at a very young age or appear in the very elderly. Primary symptoms include:

  • Lack of muscle coordination in the arms, legs, head, face and upper body
  • Progressive decline in thinking and reasoning skills, including memory, concentration, judgment and the ability to plan and organize
  • Mood disturbances, including depression, anxiety, anger, and irritability
  • Obsessive-compulsive behaviors

The last type of early-onset dementia, which is extremely rare, is Creutzfeldt-Jakob Disease (CJD). CJD is characterized by rapid neurological degeneration. It is always fatal, and death usually occurs within six months to a year of onset.

CJD belongs to a class of human and animal diseases called transmissible spongiform encephalopathies (TSEs), because the infected brain looks like a sponge. The average age of onset for CJD is 60.

“Mad Cow Disease” is the bovine equivalent of CJD (although it tends to affect younger people, with the average age of onset being 26). 

There are three types of CJD:

  • Sporadic (no known cause) – accounts for about 90% of cases
  • Inherited (family history of the disease) – accounts for 5-10% of cases
  • Acquired (transmitted by exposure to brain or nervous system tissue, usually through certain medical procedure) – accounts for less than 1% of cases

Creutzfeldt-Jakob Disease MRIThe symptoms of CJD include:

  • Rapidly progressive dementia
  • Problems with muscular coordination
  • Personality changes, including impaired memory, judgment, and thinking
  • Impaired vision
  • Insomnia
  • Depression
  • Lethargy

As CJD progresses, mental impairment becomes severe. Sufferers often develop involuntary muscle jerks (myoclonus), and they may go blind.

Eventually, they lose the ability to move and speak and become comatose. Pneumonia and other infections often occur as well, and they generally end in death.