The Many Faces of Dementia
Forgetfulness or Dementia?
What is the difference between forgetfulness and dementia?
If Aunt Clara can't remember where she put her watch, most likely she's just a bit forgetful: however, if she can't remember what numbers are or how to tell time, she may have some form of dementia.
Forgetfulness is usually temporary and doesn't affect other cognitive abilities, such as thinking and making decisions. Dementia involves severe memory loss, affects other cognitive functions and behaviors, and interferes with the person's ability to perform daily activities. Depending on the type and severity of dementia, any or all of the following mental functions may be affected:
- Thinking and reasoning - including the ability to recognize letters and numbers and to perform calculations.
- Decision making - ability to use good judgment in deciding between several alternatives or to decide what is appropriate to current circumstances. For example, a person with dementia may wear a winter jacket on a hot summer day.
- Orientation to time, date, and place - may not be aware of current time and date and may not recognize familiar people and places.
- Communication - including ability to use language appropriately and to understand spoken and written language.
- Personality - such as becoming aggressive, using profanity when not done previously, becoming anxious or suspicious, etc.
- Behavior - may behave in unusual or inappropriate ways and may have sudden mood swings with no apparent cause.
Although some forgetfulness is normal with age, the severe memory loss and other cognitive and functional effects characteristic of dementia are not inevitable. Sometimes dementia-like symptoms may be caused by lifestyle factors, such as nutritional deficiency, excessive alcohol, or sleep deprivation, or by side effects of medications. In these cases, it may be possible to reverse the effects by changing diet, reducing alcohol consumption, getting more sleep, or changing a medication.
Between forgetfulness and dementia is a condition known as mild cognitive impairment (MCI), in which a person may have impaired memory, language, or other mental function that is noticeable to others and apparent on mental tests, but doesn't interfere with the person's ability to carry on daily activities. MCI isn't considered a form of dementia because it does not interfere with activities of daily life. Having MCI increases a person's risk of developing Alzheimer's disease, especially if the main symptom is memory problems; however, not everyone who has it will progress to Alzheimer's,
Dementia often masks many underlying physical conditions. Anyone who has symptoms of dementia should consult a doctor to determine the cause and best treatment. Whatever the cause, the best defense is early diagnosis and treatment.
Diseases that Cause Dementia
The leading cause of dementia is Alzheimer's disease, a degenerative brain disorder in which brain cells die. Alzheimer's progresses slowly over a period of years, gradually destroying a person's memory and ability to learn, reason, make judgments, communicate, and go about daily activities. Later stages may also lead to changes in personality and behavior, and eventually to a need for help with dressing, personal hygiene, eating, and other basic daily activities.
Alzheimer's is believed to be caused by microscopic "plaques" and "tangles" which destroy nerve cells in the brain. The specific functional and behavioral effects of the disease can vary, depending upon which areas of the brain are being attacked. Usually the part of the brain that controls short-term memory is among the first to be damaged. Consequently, a person with Alzheimer's may be unable to remember something that happened yesterday or even five minutes ago, but may have excellent recall of things from the distant past.
Although there is no cure yet for Alzheimer's, there are treatments that may delay memory decline and alleviate some of the behavioral effects. Research is ongoing to learn what causes the "plaques" and "tangles" to form and to find new treatments to prevent or reverse damage to brain cells.
The second most common type of dementia is vascular dementia, a degenerative cerebrovascular (brain and blood vessel) disease. It develops when the flow of blood to the brain is impaired, usually due to a major stroke or a series of small strokes. When caused by a series of small strokes, or infarcts, the onset of dementia is more gradual and may be more difficult to diagnose.
Symptoms vary depending upon the number and severity of the strokes and the areas of the brain that were affected. Possible symptoms include:
- Memory problems
- Confusion, especially at night
- Lack of concentration
- Problems with communicating
- Inability to understand and follow instructions
- Difficulty with usual daily activities
- Physical symptoms of strokes, such as sudden weakness, speech impairment, shuffling steps, or loss of bladder or bowel control.
In contrast with the steady, gradual decline of Alzheimer's, vascular dementia usually progresses in steps, where sudden declines in memory and cognitive abilities are followed by a period of stability and then another sudden decline.
Since vascular dementia is closely related to diseases of the heart and blood vessels, there is some potential for preventing or treating it by managing risk factors, such as blood pressure, weight, blood sugar and cholesterol; avoiding smoking and excess alcohol, and treating underlying cardiovascular conditions. Some of the medications used to treat cognitive symptoms of Alzheimer's disease may also help people with similar symptoms due to vascular dementia.
Mixed dementia is a condition in which a person has Alzheimer's disease and another type of dementia at the same time, usually Alzheimer's disease and vascular dementia. Researchers believe that the two diseases in combination may have a greater impact on the brain than either disease alone.
Symptoms of Mixed Dementia may be the same as either Alzheimer's disease or vascular dementia, or a combination of both. Common symptoms include:
- Memory problems
- Poor concentration
- Inability to do daily tasks
There is no cure for mixed dementia. Treatment involves improving lifestyle and managing risk factors for both diseases to prevent or delay progression. Some Alzheimer's drugs, especially galantamine (Razadyne) and rivastigmine (Exelon) may be helpful. Other medications may be needed to treat stroke, high blood pressure, elevated cholesterol, or other heart problems.
Huntington's disease is a fatal degenerative brain disorder caused by mutations in one inherited gene. Onset is gradual and symptoms may vary. Common symptoms include:
- Twitches or muscle spasms
- Balance and coordination problems
- Irritability, depression, or mood swings
- Problems with memory or cognitive abilities.
Although onset may occur as early as age two, symptoms usually begin between ages 35 and 50.
Huntington's disease is hereditary. Any child of a person with Huntington's has a 50% chance of inheriting the gene. Any person who does inherit the gene will eventually develop the disease.
Lewy Body Disease
Lewy Body disease, or dementia with Lewy Bodies (DLB), is a fatal dementia which may also affect younger people, although it's more frequent in the elderly. Lewy bodies are abnormal protein deposits that form in the nerve cells of the brain. Symptoms include:
- Memory and cognitive problems similar to Alzheimer's disease
- Daytime drowsiness
- Hallucinations - often viewed as entertaining rather than fearful or threatening
- Problems with movement - shuffling, stiffness, shakiness, loss of balance, etc.
- Acting out dreams, sometimes violently
- Possible decreased tolerance, heightened sensitivity, or stronger reactions to medications.
Symptoms vary widely from person to person, and an individual's condition or symptoms may change from day to day or sometimes more frequently. Memory problems may not appear until later stages of the disease.
Parkinson's disease is a brain disorder that attacks the central nervous system. Certain nerve cells in the brain produce dopamine, a chemical which aids in smooth muscle movement. In Parkinson's patients, these cells begin to die for unknown reasons. Primary symptoms of Parkinson's disease are:
- Slow movement
- Balance problems.
People with Parkinson's usually walk with unsteady, faltering steps known as Parkinsonian Gait. The person leans forward or backward with head down and shoulders drooping, takes small shuffling steps, and may stop in the middle of a stride.
About one in five people with Parkinson's disease will develop dementia, although usually not until after age 70. Dementia symptoms in Parkinson's patients include:
- Memory problems
- Easily distracted
- Disorientation and confusion
- Slow thinking
- Lack of motivation.
Progressive Supranuclear Palsy
Progressive supranuclear palsy (PSP) is similar to Parkinson's disease in that it affects walking and balance. Unlike Parkinson's, it also affects eye movement and coordination.
The name of the disease denotes that it begins slowly and gradually worsens (progressive); it damages parts of the brain above the nuclei, which control eye movements, (supranuclear); and it causes weakness (palsy). As the name suggests, PSP affects walking, balance and eye movements.
PSP is more rare than Parkinson's. It usually affects people over age 60 but may occur as early as age 40. PSP itself is not fatal, but it can lead to life-threatening complications, such as pneumonia and swallowing problems.
- Loss of balance while walking - stiff, awkward gait; tendency to fall backward
- Personality changes - such as apathy, irritability, crankiness, and forgetfulness; may laugh or cry with no apparent reason
- Inability to focus eyes properly - blurred or double vision, inability to look downward, difficulty controlling eyelids, inability to maintain eye contact
- Slurred speech
- Difficulty swallowing food or liquids
While the cause of PSP is unknown, the symptoms result from deterioration of cells in the brainstem, cortex and basal ganglia - areas of the brain which control eye and body movements. PSP is not believed to be contagious or hereditary.
Although PSP is not fatal, there is no cure for it. Treatment focuses on managing and improving the symptoms.
Creutzfeldt-Jakob disease (CJD) is another rare degenerative brain disorder. It generally affects men and women between the ages of 50-75 years.
CJD is a prion disease. Prions are types of protein. Although they are normally harmless, infectious forms can cause CJD and other diseases. Prion diseases, which are rare and fatal, affect both humans and certain animals, including cows and sheep.
Early symptoms of CJD are similar to those of other dementias:
- Failing memory
- Behavioral changes
- Lack of coordination
- Visual disturbances.
In later stages symptoms include:
- Rapid mental deterioration
- Involuntary movements
- Weakness of extremities
There is no treatment that can cure or control CJD. Current treatment focuses on alleviating symptoms and making the patient as comfortable as possible.
Research is continuing to determine what makes a prion protein infectious and to determine exactly how CJD damages the brain and who is susceptible to the disease.
Frontotemporal dementia (FTD) is a rare disorder that involves shrinking of the brain's front (frontal lobes) and sides (temporal lobes), which control speech and personality. One form of the disease, known as Pick's disease, is caused by abnormal microscopic deposits called Pick bodies, but they're not always present in other types of FTD.
FTD has a more rapid onset than the degenerative dementias, such as Alzheimer's. There are two primary types of FTD. The first is characterized by changes in personality, judgment, planning and social functions. Behavior may be either impulsive or apathetic and includes inappropriate social interaction, such as making rude or off-color remarks. Other signs may include changes in eating habits and food preferences, an increased interest in sex, neglect of personal hygiene, and compulsive behavior. The second type of FTD is related more to language and speech problems, including both speaking and understanding speech. The behavioral symptoms may also appear in this type.
Although there is no cure or treatment that can slow the progression of FTD, there are medications that may relieve symptoms of depression and help to control behaviors that are socially unacceptable. As with all other forms of dementia, FTD requires early detection and treatment of symptoms to enable the best quality of life possible for the patient and his or her family.
Normal Pressure Hydrocephalus
A clear fluid known as crebrospinal fluid (CSF) surrounds the brain and spinal cord. It is produced in cavities in the brain called "ventricles" and circulates from one ventricle to another to cushion and protect the brain and spinal cord, provide nutrients to the brain cells, and remove waste. Normally excess fluid drains away and is absorbed elsewhere in the body.
Hydrocephalus occurs when too much CSF is present in the brain, usually because the system for draining it away is not working right. The ventricles expand to hold the fluid, causing pressure on other areas of the brain.
Normal pressure hydrocephalus (NPH) is a type of hydrocephalus that occurs in adults, usually those over age 60. It develops slowly over time. The fluid pressure may not be as high as in other types of hydrocephalus, and may even appear within the normal range on a spinal tap - hence the name "normal pressure hydrocephalus."
In most cases the cause of NPH is unknown; however, it can occur as a consequence of head injury, bleeding around the brain due to a blow to the head, stroke, meningitis, and brain tumor. It's also possible after brain surgery.
- Difficulty walking - unsteadiness, leg weakness, sudden falls, shuffling steps, etc.
- Loss of bladder control
- Decline in mental functions - slower processing and reaction to information; responses are delayed but appropriate
- Headache, nausea, and difficulty focusing eyes.
Treatment may involve surgery to insert a shunt to drain excess fluid from the brain. It usually helps more with walking and bladder control than with mental functioning. Where surgery is not recommended, treatment focuses on medications to relieve mood and behavioral problems, cope with physical problems, and improve functioning.
Wernicke-Korsakoff syndrome is a two-stage brain disorder caused by a deficiency of thiamine (vitamin B-1), which converts sugar to energy in brain cells. Without adequate thiamine the brain cells cannot produce enough energy to function. It is usually caused by alcoholism, but may also be attributed to AIDS, chemotherapy side effects, and excessively high thyroid hormone.
The first and more acute stage is known as Wernicke encephalopathy. It causes damage in lower regions of the brain known as the thalamus and hypothalamus. Symptoms of this stage include:
- Impaired vision
- Poor muscle coordination and weakness in the legs.
Korsakoff psychosis, or Korsakoff's amnesic syndrome, develops as Wernicke's symptoms pass. It is the longer-lasting, chronic stage, which results from damage to parts of the brain that control memory. Although it is a memory disorder, it also involves the heart, vascular, and nervous system.
Symptoms of Korsakoff psychosis are:
- Memory gaps and inability to form new memories
- Tendency to make up explanations for lost memories and to believe them
- Hallucinations (seeing or hearing things that are not there)
Treatment with high-dose thiamine may reverse some of the damage if the condition is diagnosed early and the person stops drinking.
The Faces of Dementia
The faces behind the mask of dementia are not "crazy" or "evil." They are the faces of real people who didn't ask for dementia, don't want it, and can't make it go away. Many live in a nightmare world of strange people and places. They cling to familiar routines and people - even if they can't remember their names - that provide some semblance of order and normalcy for them. Their lives are like a cracked mirror slowly breaking apart. As the pieces fall out one by one, the image becomes less and less distinguishable until nothing is left.
Whatever the disease or other cause of the dementia, the people who have it are still persons. In the early stages of most forms of dementia, there may be little discernible evidence of it; and simple memory aids, such as notes to themselves or reminders from family or friends may allow relatively normal lifestyles. In later stages they may have lost their memories, even their very identities, but they still have feelings and they are still capable of enjoying life in the present moment. And they can still tell when someone cares about them.
When dealing with or caring for a person with dementia, it's important to treat him or her with dignity and respect and overlook inappropriate remarks or behaviors that are beyond the person's control.
The faces of dementia are all around us. They're the faces of grandparents, aunts and uncles, husbands and wives, friends and neighbors. They're the faces of former construction workers, auto mechanics, school teachers, accountants, artists, musicians, scientists, doctors, nurses, lawyers, politicians, movie stars, bakers, plumbers, farmers, aerospace engineers, and even writers. When you see them, be patient and kind - for someday the faces of dementia may be yours and mine.