“The intuitive mind is a sacred gift, and the rational mind its faithful servant.  We have created a society that honours the servant and has forgotten the gift.” – Albert Einstein 

A reader of my weekly column recently wrote requesting I write about the dementia process known as Lewy body disease.  She notes “…please address Lewy Body Dementia in one of your excellent and informative columns.  I am sure the information could help many who are floundering like we were…”  In consideration of her request I felt it would serve the reader even more fully to once again address the broad range of dementia disorders including Lewy body disease.  In Part 1 of this two-part series the reader will be presented an overview of dementia, including its signs and symptoms, and be introduced to the more common progressive (worsening over time) forms of this all too often devastating disease.

Dementia is the loss of mental functioning (thinking, reasoning, memory) that is of such severity as to interfere with daily functioning.  It is not, itself, a specific disease, but rather a collection of signs and symptoms which may include alterations in behavior, mood and personality.  Dementia arises when areas of the brain involved in learning, decision-making, judgment, language and memory are affected by one or more of a vast array of infections or diseases.  Dementia can cause confusion, difficulty remembering people’s names, and changes in social behavior and personality.  Although memory loss often occurs in dementia, memory loss alone does not form the basis of such a diagnosis.  Most types of dementia are degenerative, non-reversible; meaning the changes in the brain that are causing the dementia cannot be stopped or turned back.  Alzheimer’s disease is by far the most common cause of progressive dementia, causing 50-60% of all dementias.  Recently, however, researchers have found that a nervous system disease, known as Lewy body disease which for years was erroneously diagnosed as Alzheimer’s, is emerging as a significant cause of dementia.  There are dozens of other known causes, most of which are thankfully rare.

Dementia is considered a late-life disorder as it tends to develop primarily in the elderly.  Approximately 5-8% of all individuals over sixty-five have some level of dementia, and this number is estimated to double every five years above that age.  It is estimated that as many as half of those in their eighties suffer from some form of dementia. 

Symptoms of dementia vary depending on the cause, and include difficulty with many areas of mental function although it usually first appears as forgetfulness.   A condition known as Mild Cognitive Impairment (MCI) is a stage between normal forgetfulness due to aging and the development of dementia.  Not everyone with MCI goes on to develop dementia.  Individuals with Mild Cognitive Impairment are oftentimes aware of their forgetfulness, and their cognitive and memory challenges do not interfere with day-to-day functioning.   Some of the symptoms of MCI include:  forgetting recent events; difficulties making decisions and problem solving; and, taking notably longer to perform more complex mental exercises.

Since the vast majority of dementias are progressive in nature, the signs and symptoms of the disorder ‘evolve’ over time.  The early symptoms of dementia include:

• Misplacing items
• Getting lost in familiar places
• General memory loss
• Difficulty coming up with the name of familiar items
• A flattening of affect accompanied by a loss of interest in previously enjoyable activities
• Changes in personality that can lead to inappropriate statements and/or behaviors
• Difficulty performing previously common mental tasks
• Inability remembering or learning new information
• Struggling with planning and organizing

As the disorder advances in severity, the symptoms become more obvious and often dramatically interfere with the person’s ability to adequately care for themselves.  Progressive symptoms may include:

• Challenges performing basic tasks
• Withdrawing from social contact
• Problems communicating effectively
• Change in sleep patterns often accompanied by multiple awakenings throughout the night
• Difficulty with motor functions
• Poor judgment with the loss of ability to recognize dangerous situations
• Difficulty reading and writing
• Forgetting events in your own life
• Agitation
• Hallucinations which can lead to aggressive behaviors
• Paranoid delusions

As the dementia process reaches final stages of severity patients can no longer:  understand language; recognize family members; and, perform even the most basic activities of daily living.  Towards the very end of this long and debilitating process most patients will be incontinent, and many will barely be able to swallow.

 Dementia most often occurs in older aged individuals.  It is uncommon for it to be diagnosed in someone under the age of sixty, and the risk of dementia increases with age.  It is estimated that approximately one-in-five cases of dementia are treatable, in that they can be either partially treated, dramatically lessening the signs and symptoms of the disease, or actually cured.  Some of these are dementias caused by hormone and/or vitamin imbalances, drug reactions and interactions, and illicit drug use and alcohol.  Severe depression, and other significant psychiatric disorders, can produce symptoms that appear to be dementia (known as pseudo- or false dementia), and is also highly treatable.  This is why it is absolutely critical that your physician be extremely thorough when making the diagnosis so as not to miss a potentially treatable cause.  Sadly, however, the vast majority of cases of true dementia cannot be cured or even significantly reversed. 

 Progressive dementias are the type of dementias that worsen over time.  They include:  Alzheimer’s disease, vascular dementia, Lewy body dementia, fronto-temporal dementia, HIV associated dementia, Huntington’s disease, and dementia pugilistica. 

Alzheimer’s disease is by far the most common cause of dementia in people over sixty-five.  Symptoms typically present after the age of sixty, although early-onset forms of the disease can appear at a younger age.  It usually progresses somewhat slowly, over years, leading to a gradual deterioration in the person’s cognitive ability.  Ultimately, the areas of the brain affected begin to no longer function properly.  Much research has been done, and aggressively continues, on this devastating disease, and although the exact causative factors are still not known two types of brain cells are commonly found to be damaged.  They are described in both professional and lay publications as ‘tangles’ and ‘plaques’.  Tangles, as the name implies, are fibrous tangles composed of a particular protein not normally found in the brain.  Plaques on the other hand are a normally harmless protein which, in the case of Alzheimer’s disease, is found clustered together. 

Vascular dementia is the second most common form of dementia.  It is caused by damage to the brain as a result of diminished blood flow to the brain secondary to some form of arterial disease and can co-exist with Alzheimer’s disease.  The onset of the signs and symptoms of vascular dementia occur rather abruptly, often following a stroke or heart attack.  They are also more common in individuals suffering from uncontrolled high blood pressure.  They can be cause by infections in the heart as well as a build-up of certain types of protein in the blood vessels in the brain.  Due to the vascular cause of this form of dementia the presentation of symptoms can be highly variable.  Some may affect just one side of the body, others may produce only memory loss, yet others confusion and mood swings.  In some forms the symptoms progressively worsen, while in others symptoms may only be temporary. 

Lewy body disease is another leading cause of dementia in older adults accounting for some twenty percent of the people diagnosed with dementia.  As with Alzheimer’s disease, abnormal clumps of proteins are found in the brain, and the likelihood of suffering from Lewy body disease increases with age.  The signs and symptoms of the disease are also very consistent with those of Alzheimer’s except that with Lewy body disease the sufferer will often experience notable fluctuations between rather clear and lucid thinking and significant confusion and visual hallucinations.  They also will have a tendency to thrash around in their sleep.

Fronto-temporal dementia is a notably less common cause and tends to occur between the ages of forty and seventy, significantly younger than in either Alzheimer’s or Lewy body disease.  In this form of dementia there is an actual erosion of nerve cells in both the frontal and temporal lobes of the brain.  As these areas are commonly associated with language, behavior and personality, the signs and symptoms of fronto-temporal dementia include:  language difficulties, problems concentrating, loss of mental acuity and frank socially inappropriate behavior. 

HIV (human immunodeficiency virus) associated dementia is a result of the extensive brain damage that occurs in advanced stages of HIV and results in difficulty concentrating, marked memory impairment, social withdrawal and apathy.

Huntingtons’ disease is an inherited disorder which results in certain nerve cells in the brain and spinal cord to virtually wither away.  The signs and symptoms of this disorder usually develop in the third and fourth decades of life.   They typically begin with depression, irritability and anxiety, but ultimately progress to severe dementia, along with great difficulty with weakness and clumsy and lumbering movements.

Dementia pugilistica, also known as “boxer’s dementia”, is caused by repeated trauma to the head.  Depending on what particular part(s) of the brain are damaged, it can cause a variability in signs and symptoms.  Poor coordination, memory difficulties, impaired speech, tremors and Parkinson’s disease can all develop as a result of such repetitive brain injury.  In dementia pugilistica the symptoms usually appear years after the traumas whereas in post-traumatic dementia, as a result of a single severe traumatic brain injury, the symptoms present acutely following the trauma and often times includes persistent memory difficulties.

In Part 2 of this series I will discuss the reversible forms of dementia, along with the risk factors, complications, prevention, treatment and resources available regarding this all too demoralizing and damaging disease.