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Lewy body dementia (LBD) affects over a million individuals in the United States. Many experts consider it the third most common cause of dementia after Alzheimer’s disease and vascular dementia, consisting of 5-10% of the total cases. Its symptoms typically appear at the age of 50 or above, and it is slightly more prevalent in men than in women.

LBD was first described in 1912 when a German doctor, Dr. Frederich H. Lewy, discovered neuronal inclusions in the brain of people with Parkinson’s disease while working in Dr. Alois Alzheimer’s laboratory. These inclusions or protein clumps, which were then named “Lewy bodies” after him, are a tell-tale sign of Lewy body dementia.

The formation and deposition of Lewy bodies occurs in the neurons of the brain portions responsible for memory, thinking, behavior, and movement. They are chiefly composed of alpha-synuclein proteins that induce chemical changes in the brain, leading to cognitive decline. Although it is abundantly present in the brain, its general function is still unknown.

What causes Lewy body dementia?

It is still unclear what causes Lewy body dementia. Researchers are learning more about its biology and the impact of genetics in order to determine possible causes and risk factors. Studies suggest that the build-up of Lewy bodies results in the loss of neurons responsible for the production of two important neurotransmitters (chemicals that conduct messages between neurons) viz, acetylcholine and dopamine. The former is crucial for memory and learning, while the latter is involved in cognition, movement, behavior, motivation, mood, and sleep.

Researchers have identified various risk factors of Lewy body dementia, among which age is the most prominent factor. Certain health conditions and diseases, particularly Parkinson’s disease and REM sleep behavior disorder, can also increase an individual’s risk of developing LBD.

Family history may also be a risk factor, but LBD is not a genetic disorder. Scientists have reported variants in three genes, APOE, SNCA, and GBA, linked with an increased LBD risk. The cause is, however, not known in most cases.

What are the Signs and Symptoms of Lewy Body Dementia?

Since LBD is a progressive disorder, it slowly develops and worsens with time. A person usually lives up to an average of five to eight years after diagnosis. However, some people may live from two to twenty years.

Similar to Alzheimer’s disease, LBD develops in stages. Symptoms are mild during the early stages, and people usually exhibit relatively normal functioning. However, As the disease progresses, movement and cognition continue to decline. At the late stage, people may become entirely dependent on others for assistance and care.

The development and progression of LBD varies greatly from individual to individual and is contingent on certain factors such as age, health, and severity of symptoms. LBD patients may not experience all the symptoms of the disease. Should a person exhibit a sudden functional or behavioral change, it should be reported to a doctor. The primary symptoms of the disease are changes in cognition, movement, sleep, and behavior.

● Cognitive Symptoms

Lewy body dementia can cause severe cognitive impairment that interferes with daily activities. LBD may seem similar to Alzheimer’s dementia in this regard, but unlike Alzheimer’s, these memory changes may not initially be apparent. Instead, they may arise with it’s progression. The cognitive symptoms include:

1. Visual hallucinations and sometimes, non-visual (auditory, olfactory, or tactile) hallucinations
2. Changes in concentration, alertness, and attention that vary throughout the day or from one day to another
3. Disorganized and unclear ideas
4. Severe cognitive impairment that often arises with any disease progression
5. Poor judgment and confusion
6. Difficulty with language and numbers

● Changes in Movement

Movement problems vary from person to person. Some people may not significantly experience these symptoms for several years, while others may develop them even in the earlier stages of LBD. These symptoms include:

1. Stiff muscles
2. Balance problems with frequent falls
3. Stooped posture
4. Tremors or shaking, particularly during rest
5. Slow movement and shuffling walk
6. Swallowing problems
7. Loss of coordination

● Changes in Sleep

Although sleep problems are widespread in LBD patients, they are generally undiagnosed. Following are the sleep changes that a person with LBD may experience:

1. REM sleep behavior disorder (which involves physically acting out dreams while sleeping)
2. Insomnia
3. Too much sleep during daytime
4. Restless leg syndrome (characterized by an overwhelming urge of moving legs when at rest)

● Behavioral Symptoms

People with LBD may experience behavioral and mood changes that may worsen with declining cognitive functioning. The symptoms include:

1. Depression and anxiety
2. Apathy and loss of motivation
3. Agitation and restlessness
4. Delusions, suspicion, and paranoia

Diagnosis of Lewy Body Dementia

Currently, there is no single test for precisely diagnosing Lewy body dementia. Since its symptoms resemble other types of dementia, such as Alzheimer’s disease and Parkinson’s disease, it becomes difficult to diagnose, particularly in the early stage. The doctors can make the diagnosis with their professional judgment of the symptoms.

Many scientists consider Lewy body dementia and Parkinson’s disease dementia to be two different interpretations of the same fundamental problems arising from the brain’s, processing of the alpha-synuclein protein. However, most experts suggest diagnosing them as distinct disorders.

The diagnosis is LBD when:

• Dementia symptoms related to LBD develop first.
• Movement changes and dementia symptoms are diagnosed simultaneously
• The symptoms of dementia emerge within one year after movement symptoms.

On the other hand, the diagnosis is Parkinson’s disease dementia should an individual, who is already diagnosed with Parkinson’s due to manifestation of movement symptoms, does not exhibit dementia symptoms until after a year or more.

However, due to the co-existence of Lewy bodies with Alzheimer’s brain changes, distinguishing LBD from Alzheimer’s disease may sometimes become challenging, particularly in the early stages.

For diagnosis of LBD, doctors may recommend a few tests such as a neurological and physical examination, blood tests to check hormone levels, assessment of mental abilities, brain scans (an MRI or CT scan), and sleep evaluation.

Alzheimer’s disease is a progressive disorder that starts and develops gradually in older people. As it progresses, the brain undergoes several changes, affecting memory, language, and thinking skills.

Brain shrinkage to a certain extent is normal in healthy aging but, surprisingly, the neurons are not lost in substantial numbers. In Alzheimer’s, however, the brain shrinks significantly due to extensive damage and neuron loss. The neurons lose the connections between them, stop functioning, and die since the disease obstructs their critical processes, including communication, metabolism, and repair.

What Parts of the Brain are Affected by Alzheimer’s?

At the outset, Alzheimer’s damages the neurons in parts of the brain which are located in the temporal lobe, including the hippocampus and entorhinal cortex, both being essential for memory. Subsequently, it deteriorates the cerebral cortex even further, affecting the frontal lobe and parietal lobe, resulting in problems with intelligence, behavior, judgment, and language. In due course, the widespread damage to many other parts of the brain leads to the gradual loss of independent functioning in people with Alzheimer’s disease., requiring full time care.

What happens to the brain with Alzheimer’s?

During Alzheimer’s progression, several cellular and molecular changes occur in the brain that can be seen microscopically in an autopsy after the patient’s death. Researchers are still trying to determine which changes cause Alzheimer’s and which changes are the results of the disease. The most prominent effects on the brain 3include the formation of beta-amyloid plaques, neurofibrillary tangles, loss of neuronal connections, and cell death.

● Amyloid Plaques

The formation of beta-amyloid proteins is a hallmark of Alzheimer’s disease. They are the minute protein particles formed by the degradation of a sizeable protein, the amyloid precursor protein.

Among its several forms, beta-amyloid 42 is the most toxic of them. The formation of this naturally occurring protein forms plaques that accumulate between the brain cells, disrupting the neuronal connection and cell function. Research is still underway to clearly understand the influence the different forms of beta-amyloid proteins have on the brain during various stages of Alzheimer’s development and progression.

● Neurofibrillary Tangles

Tau protein occurs naturally in healthy brain tissue, where it binds to and stabilizes microtubules. Microtubules are a part of the neural cell structure responsible for guiding nutrients and molecules from the cell body to both the axon and dendrites. In the Alzheimer’s brain, tau proteins form abnormal accumulations known as neurofibrillary tangles due to anomalous chemical changes. These proteins detach from microtubules and form threads by adhering to other tau molecules. Eventually, these threads join together to form tangles and disrupt neuronal communication by obstructing the cellular transportation system.

Several studies suggest a complex interplay among beta-amyloid proteins, tau proteins, and various other factors. The accumulation of abnormal tau occurs in the brain regions that control memory. Beta-amyloid, on the other hand, forms plaques between the brain cells.

● Cerebral Atrophy

Another critical Alzheimer’s-related brain change is cerebral (or brain) atrophy. With the widespread damage to neurons, connections between them are lost, eventually leading to brain shrinkage. Brain volume is reduced significantly due to extensive cerebral atrophy at the final stages of the disease.

● Chronic Inflammation

Studies suggest the role of glial cells in triggering brain inflammation. These cells keep the brain debris-free, but their build-up may cause inflammation. Microglia is a form of the glial cell responsible for initiating immune response in a healthy brain and they engulf and then degrade the toxins and wastes. In Alzheimer’s brain, however, these cells do not clean up the debris and accumulated proteins, including beta-amyloid plaques. Researchers are still trying to determine the reason for the failure of microglia to perform the debris-cleaning function in Alzheimer’s. The microglia are also involved in stimulating inflammation by releasing chemicals, further destroying the neurons they are meant to preserve.

Having a family history of Alzheimer’s disease might make you concerned about developing it yourself at an older age. In that case, you may want to undergo genetic testing to know about the probability of developing Alzheimer’s.

Multiple researchers have highlighted the link between an increased Alzheimer’s risk and a person’s genetics. Scientific findings show that individuals with first-degree relatives that have had Alzheimer’s are at a greater risk of developing the disease. The risk multiplies further if a person has more than one first-degree relative with Alzheimer’s.

Although scientific advancements, such as genetic testing, can conveniently detect hereditary diseases, the question remains, would it even be helpful to determine an individual’s risk of developing Alzheimer’s via this technique? Before we know the answer to this question, we must know about the genes and genetics of Alzheimer’s disease.

Alzheimer’s Disease and Genetics

Scientists have identified two types of genes, risk genes and deterministic genes, that are associated with late-onset and early-onset Alzheimer’s disease, respectively.

● Risk Genes

Risk genes increase the probability of Alzheimer’s development in an individual but do not guarantee the disease. These genes are associated with the most common form of dementia, the late-onset Alzheimer’s disease. Since its symptoms become apparent after the age of 65, scientists are convinced that it is not solely dependent on genetics.
Instead, other factors, including environment and lifestyle, also contribute to its development.

Researchers have discovered several risk genes. Among them, a genetic variant of the APOE (apolipoprotein E) gene on chromosome 19, APOE ε4, is the strongest indicator of an increased Alzheimer’s risk. Approximately 40-65% of people diagnosed with late-onset Alzheimer’s have this gene.

Besides APOE ε4, there are two more variants of the APOE gene viz. APOE-e2 and APOE-e3. Every individual inherits two copies of APOE genes, one from each biological parent. Inheriting a single copy of this gene increases a person’s risk. Upon inheriting two copies of this variant, the risk is augmented even further, but the disease is not guaranteed. Besides the increased risk, APOE ε4 may potentially be responsible for the onset earlier than usual.

It is important to keep in mind that not everyone with the APOE ε4 gene develops Alzheimer’s. Similarly, a person may develop it without inheriting this variant at all.

● Deterministic Genes

Deterministic genes can directly cause the disease and are responsible for early-onset familial Alzheimer’s disease. In this rare type of Alzheimer’s (which represents only 10% Alzheimer’s patients), symptoms usually appear between the 30s and mid-60s.

Any individual who inherits even one copy of these deterministic genes will develop the disorder due to their autosomal dominant pattern of inheritance. According to genetic scientists, these rare genes are found in only a few hundred extended families around the globe and account for only 1% of total Alzheimer’s cases.

Scientists have identified mutations in three genes located on different chromosomes that are responsible for causing the early-onset type:

1. Amyloid precursor protein (APP) on chromosome 21
2. Presenilin 1 (PSEN1) on chromosome 14
3. Presenilin 2 (PSEN 2) on chromosome 1

These mutations are involved in the production of disease-related abnormal proteins and the degradation of the APP protein. This protein breakdown contributes to the generation of detrimental amyloid plaques, the indicator of Alzheimer’s disease.

Genetic Testing for Alzheimer’s

Although genetic testing can detect both the APOE ε4 gene and rare Alzheimer’s genes, most healthcare professionals do not recommend them for late-onset Alzheimer’s disease. This is because the presence of the APOE ε4 gene does not guarantee the development of Alzheimer’s in an individual, weakening its position as a deciding risk factor. Genetic testing for APOE ε4 is mostly done for research purposes. However, it may be appropriate for diagnosing early-onset Alzheimer’s disease.

Scientists believe that genetic testing may never predict the disease with absolute accuracy because several other factors are also involved in both its development and progression. For instance, an individual may have an increased risk of disease development even in the absence of the APOE ε4 gene due to cardiovascular diseases, environment, or other lifestyle factors.

What to Consider Before Undergoing the Test

A person knowing about their genetic risk factors may be empowering for some while being overwhelming for others. So, before you decide to get tested for Alzheimer’s risk genes or deterministic genes, you must consider the potential emotional consequences associated with the test results.

We recommend that you have a thorough conversation with your healthcare provider or physician before being tested. Furthermore, consider consulting a genetic counselor before and after deciding to take it. The genetic counselor will help you determine which test you should take based on your family history. They will also discuss the prospective emotional impact of results and brief you about the possible consequences of the test, including the effect it might have on your employment, long-term care insurance, and future plans.

Some people with Alzheimer’s can live at home for a very long time, perhaps the rest of their lives. Others may need to be placed in assisted living, and caregivers will have to determine when that time is appropriate. It will differ for everyone.

However, taking certain precautions will help maintain a safe environment in the patient’s home.

Much of this will seem like child-proofing at the very least. It will be hard to consider and will certainly upset the patient. This is certainly dependent on what stage the person is at and how cognizant they are of their predicament.

Telephones

It is important to monitor phone calls as patients suffering from any degree of dementia and/or Alzheimer’s can be an easy target for criminals. They purchase lists of potential victims that are almost always age specific (over 65) and often utilize land lines, knowing that only seniors still use landlines with any frequency.
Even answering seemingly innocuous questions allows someone to record a “yes” or confirmation of a name that can be part of a scam.
Setting a phone to only a couple of rings so that the patient most often is
limited to outgoing calls only or simply turning off the ringer and giving the patient a mobile phone that is only to be utilized by family is another way to eliminate potential trouble.

Appliances

Lock up the ironing board, eliminate space heaters and curling irons and put a timing mechanism on the stove/oven. They really shouldn’t be cooking anything that takes more than 20 minutes so having the electrical cord of the stove go through a device that will turn off within a set amount of time can be important. Consider anything that might be a potential fire hazard.

Fireplace

Try to make fireplaces inoperable. Remove any matches, tools and firewood from the area and don’t use it even when you are there so that the habit is forgotten.

Emergency Number/Contact Information

Put a large placard near any phone that has the number for police and fire department and also the primary caregiver in addition to the home phone patient’s number and address so it is easily accessible.

Electrical Outlets

Put covers over any electrical outlets that are not in use.

Door Locks

Remove locks from any bedroom and bathroom doors so the patient cannot lock themselves in. You may want to consider door levers that are easier to operate.

Potential Weapons

Patients are prone to paranoia and fear and might hurt someone they love or even injure themselves. Remove guns, knives or anything that can be used as a weapon.

Thermostat Cover

Install a cover over the thermostat so the patient cannot accidentally turn off heat or set it too high. You can also purchase some new types of thermostats that require a password or that allow you to preset temperature parameters.

Stairs

Install two handrails for any stairs that are still being used. If a specific stair should no longer be used a door or gate will be a necessity.

Colors

Patients can sometimes relate to color better than remembering objects. A brightly colored chair might designate where you would prefer the person to sit. Similar colored rugs may guide a patient to safe paths through a house.

Alarms

Be sure smoke detectors are plentiful and operable, in addition to carbon monoxide detectors.

Spare Key

Be sure to have a spare key outside the house in case the patient accidentally locks you out or if you have to direct someone else into the home in an emergency.

Plastic Bags

A patient may accidentally smother themselves. Remove dry cleaning bags or any plastic bags if possible. Paper bags are a good option.

Computers

Eliminate or limit access to computers. They are no less dangerous than a phone for being subject to scammers.

Personal Papers

Review all paperwork. Secure anything that could be used by criminals. You might be surprised by people that have lost their homes to people that gain access to a patient’s deeds and titles.

Laundry Room

A laundry room is full of chemicals that can be dangerous.

Garage and Basement

Much like a laundry room a garage or basement can be stocked with things that can be dangerous. Keep the door to the garage locked at all times. Go a step further and hide car keys to avoid any temptation to get into the car or upset feelings when that desire is denied.

Many garages hold lawn equipment, including lawn mowers and weed eaters, which definitely should be kept off limits.

Garages sometimes have inviting work areas and lots of tools. They house sharp could-be weapons, such as shovels, axes, hoes, and rakes. Garages also store oil, paint, and other chemicals that could be deadly if ingested.

Pets

A final thing to think about are the pets in the home. Homes with pets must make adjustments to ensure safety for both the person and the pets. It is entirely possible that the person with dementia might eat cat or dog food, or mess with the litter box. For this reason, put these items in out-of-the-way places, such as the off-limits laundry room. You may have to install a doggy door to allow free access for dogs to their food or cats to the litter box.

Keep fish tanks out of reach. The combination of glass, water, electrical pumps, and potentially poisonous aquatic life could be harmful to a curious person with Alzheimer’s disease.

Also Read: Ways To Slow The Progression Of Alzheimer’s Disease