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Is Lewy Body Dementia A Genetic Disorder

Coping With Cognitive Changes

What is Lewy Body Disease?

Some medications used to treat Alzheimer’s disease also may be used to treat the cognitive symptoms of LBD. These drugs, called cholinesterase inhibitors, act on a chemical in the brain that is important for memory and thinking. They may also improve hallucinations, apathy, and delusions. The U.S. Food and Drug Administration has approved one Alzheimer’s drug, rivastigmine, to treat cognitive symptoms in Parkinson’s disease dementia. Several other drugs are being tested as possible treatments for LBD symptoms or to disrupt the underlying disease process.

Types Of Lewy Body Dementia And Diagnosis

LBD refers to either of two related diagnoses dementia with Lewy bodies and Parkinson’s disease dementia. Both diagnoses have the same underlying changes in the brain and, over time, people with either diagnosis develop similar symptoms. The difference lies largely in the timing of cognitive and movement symptoms.

In DLB, cognitive symptoms develop within a year of movement symptoms. People with DLB have a decline in thinking ability that may look somewhat like Alzheimer’s disease. But over time, they also develop movement and other distinctive symptoms of LBD.

In Parkinson’s disease dementia, cognitive symptoms develop more than a year after the onset of movement symptoms . Parkinson’s disease dementia starts as a movement disorder, with symptoms such as slowed movement, muscle stiffness, tremor, and a shuffling walk. These symptoms are consistent with a diagnosis of Parkinson’s disease. Later on, cognitive symptoms of dementia and changes in mood and behavior may arise.

Not all people with Parkinson’s disease develop dementia, and it is difficult to predict who will. Many older people with Parkinson’s develop some degree of dementia.

Caregivers may be reluctant to talk about a person’s symptoms when that person is present. Ask to speak with the doctor privately if necessary. The more information a doctor has, the more accurate a diagnosis can be.

Risk Loci For Lewy Body Dementia

The researchers performed whole genome sequencing in large cohorts of LBD patients and neurologically healthy controls. Their aims were to study the genetic architecture of the disease and produce a genetic resource for the scientific community.

Genome wide association analyses identified 5 independent risk loci. These were also replicated in an independent case-control cohort. 3 of these loci, GBA, APOE and SNCA were known risk loci of LBD. However, BIN1 and TMEM175 were newly identified risk loci. Notably, both have been implicated in other age related neurological diseases.

The BIN1 gene encodes a protein involved in endosomal trafficking. The depletion of this gene leads to increased amyloid beta production and promotes the propagation of tau pathology. The pathogenic impacts of this gene in LBD appear to be the same as in AD. Thus, mitigation of BIN1-mediated endosomal dysfunction could have therapeutic implications in both neurodegenerative diseases.

On the other hand, TMEM175 is a known Parkinsons disease risk locus. Deficiency of this gene impairs lysosomal function and leads to increased deposition of phosphorylated alpha-synuclein. The identification of this gene underscores the role of lysosomal dysfunction in the pathogenesis of Lewy body diseases.

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Medications For Sleep Disorders

Sleep problems may increase confusion and behavioral problems in people with LBD and add to a caregiver‘s burden. To help alleviate sleeplessness in people with LBD, a physician can order a sleep study to identify any underlying sleep disorders such as sleep apnea, restless leg syndrome, and REM sleep behavior disorder.

REM sleep behavior disorder, a common LBD symptom, involves acting out one’s dreams, leading to lost sleep and even injuries to sleep partners. Clonazepam , a drug used to control seizures and relieve panic attacks, is often effective for the disorder at very low dosages. However, it can have side effects such as dizziness, unsteadiness, and problems with thinking. Melatonin, a naturally occurring hormone used to treat insomnia, may also offer some benefit when taken alone or with clonazepam.

Excessive daytime sleepiness is also common in LBD. If it is severe, a sleep specialist may prescribe a stimulant to help the person stay awake during the day.

Some people with LBD may have difficulty falling asleep. If trouble sleeping at night persists, a physician may recommend a prescription medication to promote sleep. It is important to note that treating insomnia and other sleep problems in people with LBD has not been extensively studied, and that treatments may worsen daytime sleepiness and should be used with caution.

Lewy Body Dementia Symptoms

MEDICAL SCHOOL: Lewy body dementia,What to know? in 2020 ...

How can the presence of Lewy body dementia be recognized? The disease is especially;challenging to diagnose;due to its;laundry list of symptoms that can vary on an individual basis.;Early Lewy body dementia symptoms are commonly;confused with similar symptoms found in other brain disorders;like Alzheimers disease. LBD can occur alone or along with Alzheimers or Parkinsons disease.

Common LBD Symptoms:

Due to;significant changes in the part of the nervous system that regulates automatic functions, such as those of the heart, glands and muscles, people with Lewy body dementia might also experience any of the following:

  • blood pressure problems
  • sensitivity to heat and cold
  • sexual dysfunction

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Dlb Genetics Has Remained Largely Elusive

Despite the fact that we now know that genetics plays a role in the disease, genes that cause DLB are still to be identified. In comparison with AD and PD, we know far less about the genetic basis of DLB. There are multiple reasons for this. Firstly, DLB is difficult to diagnose, as phenotypic overlaps with other neurodegenerative diseases can diminish chances of an accurate diagnosis. The lines between different neurodegenerative diseases can often be blurred, and a patient may show features of one or several other diseases, both ante and post mortem. Additionally, DLB is not as frequently recognized as a disease compared to other well-known disorders, such as AD. Both factors result in a substantial rate of mis- and under-diagnosis. This has hindered the collection of large cohorts of cases whose diagnosis is certain, and as a consequence, has limited large-scale genetic analyses. Furthermore, as the disease is age-related, and typically occurs in those aged 65 or older, the likelihood of gathering biomaterials from multiple family members for genetic testing of familial DLB is small. This, coupled with the fact that families with DLB are rare, has limited the understanding of Mendelian DLB genetics. Although still a prevalent cause of disease in the elderly, DLB is less common than diseases such as AD and PD, and hence cohorts will generally be smaller for this disease.

Table 1 Representative examples of genetic studies conducted in sporadic DLB

Is There Treatment Available

At present there is no cure for Lewy body disease. Symptoms such as depression and disturbing hallucinations can usually be reduced by medication. However, medications to relieve hallucinations may increase muscle tremors and stiffness. Conversely, anti-Parkinson drugs may make hallucinations worse.

Emerging evidence suggests that cholinesterase inhibitor drugs may be quite helpful for some people with this condition.

People with this form of dementia are very sensitive to the side effects of neuroleptic drugs such as antipsychotic medications. It is essential all medications are supervised by a specialist to avoid these severe side effects.

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Can Imaging Tests Diagnose Lewy Body Dementia

Imaging tests, such as computed tomography or magnetic resonance imaging , are done to rule out other causes of dementia such as brain tumors, brain bleeds, stroke, hydrocephalus or other structural causes. Imaging studies for Lewy body dementia are usually normal. The only way to make an absolute diagnosis of LBD is by examining the brain at autopsy.

Medications For Movement Symptoms

What is Lewy body dementia and how is it treated?

LBD-related movement symptoms may be treated with a Parkinson’s medication called carbidopa-levodopa . This drug can help improve functioning by making it easier to walk, get out of bed, and move around. However, it cannot stop or reverse the progress of the disease.

Side effects of this medication can include hallucinations and other psychiatric or behavioral problems. Because of this risk, physicians may recommend not treating mild movement symptoms with medication. If prescribed, carbidopa-levodopa usually begins at a low dose and is increased gradually. Other Parkinson’s medications are less commonly used in people with LBD due to a higher frequency of side effects.

A surgical procedure called deep brain stimulation, which can be very effective in treating the movement symptoms of Parkinson’s disease, is not recommended for people with LBD because it can result in greater cognitive impairment.

People with LBD may benefit from physical therapy and exercise. Talk with your doctor about what physical activities are best.

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Risk Genes For Dementia With Lewy Bodies

Several gene variants have recently been linked to a higher risk of DLB. The APOE e4 variant is thought to be the strongest genetic risk factor for DLB, as it is for Alzheimer’s disease.

Variants in two other genes, glucocerebrosidase and alpha-synuclein , also affect the risk of a person developing DLB.

Alpha-synuclein is the main protein within Lewy bodies, which are found in both DLB and Parkinson’s disease. The GBA and SNCA genes are also both important in Parkinson’s disease.

It is not surprising that the gene variants associated with DLB also play a role in Alzheimer’s disease and Parkinson’s disease . This is because the symptoms of DLB overlap with those of both Alzheimer’s disease and Parkinson’s, and the diseases share some common underlying features.

Cognitive Symptoms Of Lewy Body Dementia

LBD causes changes in thinking abilities. These changes may include:

  • Visual hallucinations, or seeing things that are not present. Visual hallucinations occur in up to 80 percent of people with LBD, often early on. Nonvisual hallucinations, such as hearing or smelling things that are not present, are less common than visual ones but may also occur.
  • Unpredictable changes in concentration, attention, alertness, and wakefulness from day to day and sometimes throughout the day. Ideas may be disorganized, unclear, or illogical. These kinds of changes are common in LBD and may help distinguish it from Alzheimer’s disease.
  • Severe loss of thinking abilities that interfere with daily activities. Unlike in Alzheimer’s dementia, memory problems may not be evident at first but often arise as LBD progresses. Other changes related to thinking may include poor judgment, confusion about time and place, and difficulty with language and numbers.

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Behavioral And Mood Symptoms Of Lewy Body Dementia

Changes in behavior and mood are possible in LBD and may worsen as the persons thinking abilities decline. These changes may include:

  • Depression
  • Apathy, or a lack of interest in normal daily activities or events and less social interaction
  • Anxiety and related behaviors, such as asking the same questions over and over or being angry or fearful when a loved one is not present
  • Agitation, or restlessness, and related behaviors, such as pacing, hand wringing, an inability to get settled, constant repeating of words or phrases, or irritability
  • Delusions, or strongly held false beliefs or opinions not based on evidence. For example, a person may think his or her spouse is having an affair or that relatives long dead are still living.
  • Paranoia, or an extreme, irrational distrust of others, such as suspicion that people are taking or hiding things

How Can A Person Diagnosed With Lewy Body Dementia Live The Best Life Possible

Dementia with Lewy Bodies (Including Parkinsons Disease ...

First, its important to interact with others as much as possible. Plus exercise and eat a healthy diet to keep mind and body as strong as possible.

Use music and aromatherapy to reduce anxiety and improve mood.

Other things you can do involve seeking the help and assistance of others. Therapists can improve the quality of life of someone living with Lewy body dementia. Therapists include:

  • Physical therapists: These therapists can help improve physical strength, flexibility, balance and walking mechanics.
  • Occupational therapists: These therapists can teach skills and methods to maintain your ability to perform activities of everyday life and remain independent.
  • Speech therapists: These therapists can improve swallowing difficulties and ability to speak more clearly.

Persons with Lewy body dementia and their families can also benefit from counseling with a psychotherapist. This counselor teaches how to manage emotional and behavioral problems. Finally, joining a support group there are support groups for persons with LBD and for the caregivers of persons with LBD. Support groups help solve day-to-day problems and frustrations through sharing similar experiences. See the resource section of this article for links to support groups.

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What Is Lewy Body Dementia Causes Symptoms And Treatments

On this page:

Lewy body dementia is a disease associated with abnormal deposits of a protein called alpha-synuclein in the brain. These deposits, called Lewy bodies, affect chemicals in the brain whose changes, in turn, can lead to problems with thinking, movement, behavior, and mood. Lewy body dementia is one of the most common causes of dementia.

LBD affects more than 1 million individuals in the United States. People typically show symptoms at age 50 or older, although sometimes younger people have LBD. LBD appears to affect slightly more men than women.

Diagnosing LBD can be challenging. Early LBD symptoms are often confused with similar symptoms found in other brain diseases or in psychiatric disorders. Lewy body dementia can occur alone or along with other brain disorders.

It is a progressive disease, meaning symptoms start slowly and worsen over time. The disease lasts an average of five to eight years from the time of diagnosis to death, but can range from two to 20 years for some people. How quickly symptoms develop and change varies greatly from person to person, depending on overall health, age, and severity of symptoms.

In the early stages of LBD, symptoms can be mild, and people can function fairly normally. As the disease advances, people with LBD require more help due to a decline in thinking and movement abilities. In the later stages of the disease, they often depend entirely on others for assistance and care.

What Is Dementia With Lewy Bodies

Lewy body dementia is often associated with Alzheimers or Parkinsons. Indeed, it can occur in conjunction with and worsen these diseases. In the case of Parkinsons, for example, Lewy body deposits are found in the brain stem. However, as todays feature study will show, DLB is also, in fact, its own neurodegenerative condition with its own genetic makeup.

In DLB, Lewy bodies can be found in many areas of the brain, such as the cerebral cortex, hippocampus, and midbrain. Dementia with Lewy bodies accounts for 1015% of dementia cases, making it second on the list of the most common progressive dementias, with Alzheimers dementia being, by far, the most common. Lewy body dementia, like Alzheimers, is an incurable progressive dementia, which means it will continue to worsen over time and until death. My dad died about five or so years after the diagnosis, and his last years were not great.

Before we dig into todays study, lets review what else weve learned about dementia.

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Wait So What Is Parkinsonism

Parkinsonism refers to the motor symptoms that are typically associated with PD, such as tremors, stiffness, and walking/balance problems. Both PD and LBD are forms of Parkinsonism, meaning that PD patients and LBD patients may experience these motor symptoms.2 Because the Parkinsonism motor symptoms of PD and LBD can be very similar, it can be difficult to differentiate between the two conditions.

Can You Prevent Lewy Body Dementia

Parkinson’s Disease – Lewy Body Disorders: Carol Persad

If you have a family history of Lewy body dementia or Parkinson’s disease, it’s understandable to be concerned about developing Lewy body dementia. Like other types of dementia, there’s not a guaranteed way to completely prevent Lewy body dementia. However, understanding the factors that increase the risk helps us identify opposing strategies that can decrease this likelihood, and these strategies are generally connected with better physical health, as well.

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Is Lewy Body Dementia Hereditary & What Is The Life Expectancy Of A Person With Lewy Body Dementia

Most patients suffering from Lewy Body Dementia prefer visiting a primary care physician at the initial stage. Diagnosing the disorder can be a real problem if the symptoms grow severe.

There are many families that have been affected by Lewy Body Dementia . However, it has been seen that very few individuals and even medical professionals are aware of the diagnostic criteria and symptoms. Some even dont have the idea whether or not the disease exists. Therefore, you should know about Lewy Body Dementia not only to save yourself but also your loved ones, families and friends. Lewy body dementia is a type of progressive dementia that comes only after Alzheimers disease dementia.

Managing Sleep Disorders In Lewy Body Dementia

Sleep problems may increase confusion and behavioral problems in people with LBD and add to a caregiver’s burden. A physician can order a sleep study to identify any underlying sleep disorders such as sleep apnea, restless leg syndrome, and REM sleep behavior disorder.

REM sleep behavior disorder, a common LBD symptom, involves acting out one’s dreams, leading to lost sleep and even injuries to individuals and their sleep partners. Clonazepam, a drug used to control seizures and relieve panic attacks, is often effective for the disorder at very low dosages. However, it can have side effects such as dizziness, unsteadiness, and problems with thinking. Melatonin, a naturally occurring hormone used to treat insomnia, may also offer some benefit when taken alone or with clonazepam.

Excessive daytime sleepiness is also common in LBD. If it is severe, a sleep specialist may prescribe a stimulant to help the person stay awake during the day.

Some people with LBD have difficulty falling asleep. If trouble sleeping at night persists, a physician may recommend a prescription medication. It is important to note that treating insomnia and other sleep problems in people with LBD has not been extensively studied, and that treatments may worsen daytime sleepiness and should be used with caution. Sleep problems can also be addressed by avoiding lengthy naps, increasing daytime exercise, and avoiding caffeine, alcohol, and chocolate late in the day.

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